UNIVERSITY  OF  CAUFORNIA   SAJj,  Pj^||jj| 

3  1822  00502  8758 


(^  LIBRARY 


V, 


UNIVERSITY  OF 
CALIFORNIA 

SAN  DIEGO 


presented  to  the 
UNIVERSITY  LIBRARY 
UNIVERSITY  OF  CALIFORNIA 
SAN  DIEGO 

by 


Dr.  E.  H.  Christopher son 


42< 


^^O--^ 


h/h 


> ,-       *— 


UNIVERSITY  OF  CALIFORNIA   SAN  DIEGO 

lllllllllllllllliiiiiii  III  mill 


3  1822  00502  8758 


DIAGNOSTICS 


or 


THE  FUNDUS  OCULI 


BY 


EDWARD   L.   OATMAN,  M.  D. 

SURGEON  MANHATTAN  EYE,  EAR  AND  THROAT  HOSPITAL  AND  BROOKLYN  EYE  AND  EAR  HOSPITAL, 

NEW  YORK.  CONSULTING  OPHTHALMIC  SURGEON  NYACK  HOSPITAL 

AND  ST.  MARY'S  HOSPITAL,  WATERBURY,  CONN. 


COMPRISING 

ONE  VOLUME  OF  TEXT 

WITH  TWO  HUNDRED  AND  THIRTY-FOUR  ILLUSTRATIONS 

AND  FOUR  COLORED  PLATES 

AND 

TWO  PORTFOLIOS 

CONTAINING  SEVENTY-NINE  STEREOGRAMS  AND 

EIGHT  DIAGNOSTIC  CARDS 


TROY,  NEW  YORK 
THE   SOUTHWORTH   COMPANY 
1920 


Copyright,  by 

THE   SOUTHWORTH   COMPANY, 

Trot.  N.  Y. 

(All  rights  for  all  countries  reserved.^ 


PREFACE. 

In  the  Diagnostics  of  the  Fundus  Oculi,  the  author  seeks  to  present 
his  subject  in  a  manner  serviceable  to  the  ophthalmologist,  the  neurologist 
And  the  general  practitioner,  as  well  as  to  the  teacher  and  the  student. 
It  is  hoped  that  the  ophthalmologist  may  find  in  the  text,  with  its  accom- 
panying figures,  some  features  that  will  meet  with  his  approbation,  and 
that  the  stereographic  reproductions  of  the  fundus  will  recall  to  his  mind 
cases  encountered  in  his  own  practice.  There  are  many  diseased  condi- 
tions of  the  fundus  with  which  the  general  practitioner  should  be  familiar, 
and  it  is  believed  that  the  stereograms  accompanying  this  volume  will 
aid  him  greatly  in  their  recognition.  They  are  all  reproductions  of  actual 
cases  and  in  each  instance  the  fundus  conditions  have  been  depicted  with 
every  effort  possible  toward  accuracy.  The  rarer  examples,  such  as  those 
of  maculo-cerebral  degeneration  and  retinitis  punctata  albescens,  are 
hardiv  necessary  in  his  ordinary  practice,  but  it  would  seem  highly  desir- 
able and  almost  essential  that  he  should  be  able  to  call  to  his  aid  in 
diagnosis  such  common  fundus  pictures  as  those  presented  by  chois.ed  disc, 
albuminuric  retinitis  and  optic  atrophy,  with  others  less  common,  perhaps, 
as  his  knowledge  of  fundus  diseases  and  his  facility  in  the  use  of  the 
ophthalmoscope  increase.  The  teacher  will,  it  is  hoped,  find  the  stereo- 
grams of  advantage  as  substitutes,  when  necessary,  for  actual  cases  in  his 
clinical  lectures,  and  they  can  hardly  fail  to  be  of  a  similar  service  to  the 
student  of  ophthalmology.  It  is  not  without  some  misgivings  that  a 
work  of  this  character  is  presented  to  the  medical  profession.  Many  ex- 
tensive works  on  diseases  of  the  fundus  have  preceded  it,  as  have  also 
treatises  more  profound  upon  its  pathology.  It  has  been  the  endeavor  of 
the  author  to  combine  both  these  features  in  a  work  sufficiently  con- 
densed for  agreeable  study.  The  collection  of  cases  and  specimens  has 
extended  over  a  period  of  a  number  of  years  and,  in  his  efforts  in  this 
direction,  the  author  has  to  thank  many  of  his  colleagues  who  have  so  kindly 
given  their  aid  and  to  whom  reference  is  made,  it  is  hoped  in  all  instances, 
in  the  text.  In  the  preparation  of  the  pathological  specimens,  his  thanks 
are  especially  due  to  Dr.  James  Ewing  and  Dr.  Henry  T.  Brooks,  and  he 
has  also  to  thank  Dr.  Alfred  Braun  for  his  kindly  criticism  of  the  color 
tints,  as  well  as  Dr.  II.  W.  Wootton  for  suggestions  in  regard  to  the  text. 

E.  L.  Oatman. 

40  Fast  4 1st  Street. 


Digitized  by  the  Internet  Archive 

in  2008  with  funding  from 

IVIicrosoft  Corporation 


http://www.archive.org/details/diagnosticsoffun01oatm 


CONTENT  OF  PART  I. 

Chapter  I. 
EXAMIXATIOX  OF  THE  EYE. 

PAGE 

Thk  Ophthalmoscope   1 

Use  of  the  Ophthalmoscope: 

Direct  Method 3 

Indirect  Method 4 

Focal  or  Oblique  lLLr\nNATiON 5 

Transillunhnation   7 

DicirrAL  Tonometry 9 

Instrumental  Tonometry 10 

Chapter  II. 
THE  NORMAL  FUNDUS. 

Blond  Fundus 1 

Sclera  1 

Suprachoroidea   1 

Choroid    1 

Retina   1 

Retinal  Reflexes — Negroid  Fundus 14 

The  Retinal  Vessels 15 

Pulse  Phenomena 16 

The  Optic  Nerve 17 

Anatomy    1  «^ 

Chapter  III. 
CONGENITAL  ANOMALIES. 

COLOBOMA  OF  THE  CHOROID 20 

COLOBOMA  OF  CHOROID  AND  Ol'IlC  NeRVI', 21 

Pathogenesis    22 

V 


vi  CONTENTS. 

PAGE 

COLOBOMA  OF  THE  MaCULA 23 

Diagnosis    24 

COLOBOMA  OF  OpTIC  NeRVE  AND  NeRVE  ShEATH 24 

Diagnosis    26 

Inferior  Optic  Crescent  (Fuchs'  Coi.oboma)  26 

Opaque  Nerve  Fibers  (Medullated  Nerve  Fibers)   27 

Diagnosis    29 

Prepapii.t.ary  Retinal  Artery 29 

Diagnosis 30 

Pseudoneuritis    30 

Persistent  Hyaloid  Artery — Persistent  Canal  of  Cloquet.  .  32 

Diagnosis   : 33 

Albinism 33 

Diagnosis 34 

Punctate  Condition  of  the  Fundus  (Gunn's  Dots)  35 

Diagnosis 36 


Chapter  IV. 
AFFECTIONS  OF  THE  CHOROID. 

Exudative  Choroiditis 37 

Pathology 37 

Choroiditis  Disseminata,  Exudative  Stage 37 

Diagnosis 39 

Choroiditis  Disseminata,  Exudative  Stage 40 

Symptoms    41 

Diagnosis 41 

Central  Exudative  Choroiditis,  Exudative  Stage 42 

Diagnosis 43 

Choroiditis  Disseminata,  Cicatricial  Stage 43 

Repair    44 

Diagnosis 45 

Central  Choroiditis,  Commencing  Cicatrization 45 

Diagnosis 46 

Chorioretinitis  Pigmentosa;  Retinitic  Optic  Atrophy 46 

Diagnosis 47 


CONTENTS.  vn 

PAGE 

7  UBERCULOSIS  OF  THE  CHOROID 48 

Diagnosis 51 

PVom   Miliary  Tuberculosis    51 

From   Conglomerate  Tubercle   51 

From   Septic  Retinitis 52 

From   Focal  Tuberculosis  of  the  Choroid 52 

Diffuse  Chorioretinitis,  Atrophic  Stage 52 

Vascular  Degeneration  of  the  Choroid 54 

Diagnosis    55 

Atrophy  of  the  Choroid — Acquired  Albinism 55 

Diagnosis 57 

Senile  Changes  in  the  Fundus — Colloid  Excrescences — Mac- 
ular Degeneration — Arteriosclerosis 57 

Diagnosis 59 

Rupture  of  the  Choroid 59 


Chapter  V. 

SCLEROSIS  OF  THE  RETINAL  VESSELS  AND   RETINAL 

HExMORRHAGE. 

Diffuse  Retinal  Arieriosclerosis,  Primary  Stage 62 

Etiology   63 

Diagnosis 64 

Adv  \Nci:i)  ScLicRosis  OF  Retinal  Vessels * 65 

Diagnosis 66 

Morbid  Anatoiny 68 

Arteriosclerosis  and  Optic  Neiritis 69 

Diagnosis 69 

Retinal  Hemorrha(;e  in  Ar'terioscli:rosis 70 

Retinal  Hi:.morrhage 71 

Etiology  of  Retinal  Hemorrhage  in  (jeneral 7.3 

Arteriosclerosis 73 

Anemia    74 

Chlorosis 74 

Malaria    74 

Syphilis    74 

Stasis    74 

Natal  Retinal  Hemorrhage 75 


Viii  CONTENTS. 

PAGE 

Prerktixal  or  Subhvalotd  Hemorrhage 76 

Intravitreous  Hemorrhage 77 


Chapter  VI. 

ANGIOPATHIC  RETINITIS. 

Angiopathic  Retinitis  in  General 80 

Angiopathic  Albuminuric  Retinitis 81 

Precursory  Stage 81 

Diagnosis '. 82 

Developed  Angiopathic  Albuminuric  Retinitis 82 

Symptoms    82 

History  and  Course 84 

Pathology    85 

Acute  Albuminuric  Retinitis 86 

Exudative  Stage    88 

Diagnosis 89 

Degenerative  Stage    90 

Hemorrhagic  Albuminuric  Retinitis 90 

Angiopathic  Albuminuric  Neuroretinitis 90 

Diagnosis 91 

Atrophic  Stage,  Retlnltic  Optic  Atrophy 92 

Angiopathic  Diabetic  Retinitis 94 

Central  Punctate  Diabetic  Retinitis  94 

Hemorrhagic  Diabetic  Retinitis 95 

Angiopathic  Leukemic  Retinitis 96 

Leukemic  Neuroretinitis 96 

Hemorrhagic  Leukemic  Retinitis 98 

Retinitis  in  Pernicious  Anemia 98 

Angiopathic  Syphilitic  Rp:tinitis 100 

Retinitis  Circinata 101 


CONTENTS.  IX 

Chapter  VII. 
CLOSURE  OF  THE  RETINAL  VESSELS. 

PAGE 

Closure  of  the  Central  Artery 105 

Ophthalmoscopic  Appearance    105 

Interpretation  of  the  Ophthalmoscopic  Picture 106 

Foveal  Red  Spot 107 

Retinal  Vessels 108 

Granular  Current    Ill 

Hemorrhage    Ill 

Etiology    113 

Embolism 113 

Closure  of  Central  Artery  wvvu  Visible  Collateral  Circula- 
tion     115 

Etiology  (continued) 

Angiospasm    .' 119 

Thrombosis 122 

Arteriosclerosis   124 

Unusual  Causes    125 

Anatomical  Characters 126 

Clinical  History 132 

Diagnosis    133 

Occlusion  Atrophy 133 

Retinal  Atrophy  following  Closure  of  Central  Artery  ....  134 

Branch  Artery  Closure    134 

Diagnosis    136 

Closi"re  of  the  Central  Retinal  Vein ' 13S 

Diagnosis 14(1 

Etiology    140 

Oksi  ruction  of  Central  Vein  with  Neltritis 142 

Anatomical  Characters  of  Closure  of  Central  Vein 143 

Thrombosis    147 

Chnical  History 150 

Complications    151 

Closure  of  Venous  Branch 155 

post-thrombotic  cliancjes   156 

Obstruction  of  Both  Central  Vessels 156 


X  CONTENTS. 

Chapter  VIII. 
DETACHMENT  OF  THE  RETINA. 

PAGE 

Serous  Detaciimext . 159 

Incipif  NT  Detachment 160 

Diagnosis 160 

Symptoms  of  Retinal  Detachment 161 

Ophthalmoscopic  Picture  of  Recent  Detachment 163 

Differenhation  of  Retinal  Detachment 1  '4 

Reattachment  of  the  Retina 1 55 

Old  Detachment  of  the  Retina 166 

Symptoms  of  Retinal  Detachment  . 167 

Prognosis    168 

Etiology    168 

Exudative  Theory 169 

Myopia,  Mechanical  Theory 170 

Hemorrhagic  Theory 170 

Vitreous  Detachment 171 

Retraction  Theory 17  ' 

Diffusion  Theory 173 

Hyperemia  Theory 174 

Iridocyditis    174 

Traumatism    175 

Unusual  Causes 176 

Chapter  IX. 

PEGENERATIONS  OF  THE  RETINA. 

Rf.tixitis  Pigmentosa 1 79 

( )phthalmoscopic  Picture 180 

Diagnosis 181 

Retini'iis  Pigmentosa,  sine  Pigmento 181 

Atrophia  Gyrata  Choroid.?:  et  Retin.t; 181 

Course  of  Retinitis  Pi(iNH-:xrosA 1 83 

Complications    183 

Etiology    1  £^ 

Diagnosis 185 

Anatomical  Characters 186 


CONTENTS.  XI 

PACK 

Retinu  IS  Punctata  Albescens 1 89 

Ophthalmoscopic  Picture 190 

Macui.ocerehral  Degeneration 191 

Ophthalmoscopic  Picture 193-194 

Pathologic  Anatomy 195 

Etiology    196 

Differentiation    197 

From   Hereditary  Macular  Anomaly 197 

Prom   Retinitis  Pigmentosa 197 

From   Amaurotic  Family  Idiocy 198 

From   Central  Chorioretinitis 199 

From   Unclassified  Cases 199 

Amai'rotic  Family  Idiocy  (Tay's  Disease) 200 

F'tiology   201 

Morbid  Anatomy 202 

Ophthalmoscopic  Picture 203 


Chapter  X. 

MISCELLANEOUS  AFFECTIONS  OF  THE  RETINA. 

Cyanosis  Retin.^ 204 

Ophthalmoscopic  Picture 205 

Diagnosis 205 

Traumatic  Edema  (Commotio  Retin.^)   205 

Ophthalmoscopic  Picture 207 

r>iagnosis 207 

Toxic  Amaurosis  and  Amhi  yopia 208 

From   Wood  Alcohol 208 

From   Chronic  Alcoholism 209 

From  Tobacco 209 

From    Iodoform    210 

FVom   Quinine    210 

From   Salicylic  Acid    210 

From   Less  Frequent  Causes 210-21  1 

Retinitis  Prolh  erans 211 

Ophthalmoscopic  Picture 212 


XII  CONTENTS. 

PAGE 

Retinitis  Striata  and  Angioid  Streaks  in  Retina -  213 

Ophthalmoscopic  Picture 214 

Metastatic  Retinitis  (Endogenous  Ophthalmitis)   215 

Pathology 216 

Septic  Retinitis  of  Roth 217 

Pathology    218 

Diagnosis 218 


Chapter  XI. 
OPTIC  NEURITIS  AND  OPTIC  ATROPHY. 

Optic  Neuritis  and  Choked  Disc ^ 220 

Ophthalmoscopic  Picture 222-224 

Diagnosis    222—224 

Neuritis  in  Nasal  Sinus  Disease 225 

Symptoms    226 

Ophthalmoscopic  Picture 227 

The  Optic  Nerve  in  Cerebral  Sinus  Thrombosis 228 

Ophthalmoscopic  Picture 230 

Optic  x\trophy 231 

Simple,  in  Tabes   231 

Ophthalmoscopic  Picture 233 

In  Multiple  Sclerosis 233 

Ophthalmoscopic  Picture 234 

In  Other  Diseases  of  the  Nervous  System 235-236 

Neuritic  Optic  Atrophy 236 

Ophthalmoscopic  Picture 237 

Differentiation  of  Simple,  Neuritic  and  Retinitic  Atrophy 

AND  Glaucoma 238 

Compression  of  Optic  Nerve  in  Arteriosclerosis 239 


Chapter  XII. 

MYOPIA. 

Axial  Myopia 244 

Etiology  244 


CONTENTS.  XI 11 

PAGE 

Non-progressive — Ophthalmoscopic  Picture 246 

Anatomic  Changes    246 

Progressive  Myopia  248 

Ophthalmoscopic  Picture — 249 

Curvature  Myopia  249 

Index  Myopia 250 


Chapter  XIII. 
GLAUCOMA. 

Primary  Glaucoma 251 

Prodromal  Stage    251 

Acute  InHammatory  Stage    251 

Habitus  Glaucomatosus 253 

Glaucoma   Absolutum    253 

Glaucoma    Degenerativum    254 

Glaucoma   Fulminans    254 

Chronic  or  Sub-acute  Glaucoma 254 

Simple  Glaucoma    254 

Hemorrhagic  Glaucoma    255 

Ophthalmoscopic  Picture  of  Glaucoma 255 

Etiology  of  Primary  Glaucoma 257 

Secondary  Glaucoma    260 

Morbid  Anatomy   262 

Buphthalmus    264 

Ophthalmoscopic  Picture  and  Microscopic  Examination  of 
So-called  Hemorrhagic  (jlaucoma  in  Advanced  Arterio- 
sclerosis      265 

Diagnosis  of  Glaucoma  in  Genera i 266 

From  Iridocyclitis  and  Iritis 26-8 


Chapter  XIV. 
I\  I  RAOCULAR  AND  ORBITAL  TUMORS. 

Sarcoma  of  i hi-.  Ciiokoid 270 

Morbid  Anatomy  and  Varieties 272-273 


XIV  CONTENTS. 

PAGE 

Ophthalmoscopic  Picture 274 

Diagnosis   274 

From  Metastatic  Carcinoma    277 

Glioma  of  the  Retina 278 

Ophthalmoscopic  Picture 279 

Morbid  Anatomy 280 

Cryptoglioma 281 

pseudoglioma   282 

Ophthalmoscopic  Picture 283 

Diagnosis   283-286 

Tumors  of  the  Ciliary  Body 287 

Sarcoma    287 

Ophthalmoscopic  Picture  of  Sarcoma  of  Choroid  and  Ciliary 

Body    287 

Less  Frequent  Tumors  of  Ciliary  Body 288 

Tumors  of  the  Orbit    289 

Tumors  of  the  Lachrymal  Gland 291 

Tumors  of  the  Optic  Nerve 291 

Tumors  of  the  Optic  Nerve  Causing  Optic  Atrophy  and  Sec- 
ondary Hypermetropia 292 

Diagnosis  of  Tumor  of  the  Optic  Nerve 293 


LIST  OF  ILLUSTRATIONS. 

FIGURE                                                                                                                                                                       OPPOSITE  l'A(.t 

1  Ophthalmoscope  (obverse)    2 

2  Electric  ophthalmoscope 2 

3  Ophthalmoscope   ( reverse)    2 

4  Ophthalmoscopic  examination,  indirect  method 5 

5  Oblique  focal  illumination 5 

6  Normal  fields  of  vision 6 

7  Skeel's  perimeter    6 

8  Duane's  tangent  plane 7 

9  Right  homonymous  hemianopsia 7 

10  Bitemporal  hemianopsia   7 

1 1  .  Binasal  hemianopsia    7 

12  Horizontal  hemianopsia    8 

13  Wiirdemann's  transilluminator    8 

14  A  sarcoma  not  demonstrable  by  transillumination 8 

15  Digital   tonometry    10 

1 6  Schiotz  tonomet;!r 10 

1 7  The  choroid 12 

1 8  The  retinal  epithelium 12 

19  The  layers  of  the  retina 13 

20  .    The  retina  at  the  macula 13 

21,  22,  23    Normal  discs  with  physiologic  excavations 18 

24  Coloboma  of  upper  lid  with  coloboma  of  choroid 20 

25,  26      Embryology  of  Coloboma  of  the  choroid 22 

27  Coloboma  of  choroid    25 

28  Coloboma  of  choroid    25 

29  Coloboma   of  optic  nerve  and  sheaths 25 

30  Coloboma   of  optic  nerve  and  sheaths ]\ 

31  Pseudoneuritis    ]\ 

32  Suppuratixe   choroiditis    37 

33  Choroiditis,   stage  of  exudation 37 

34  Choroiditis,   stage  of  exudation M 

35  Choroiditis,    formation  of  granulation  tissue 37 

36  Choroiditis,   stage  of  cicatn/ation    37 

37  Choroiditis,   advanced  cicatrization 37 

38  Choroiditis   hyperplastica    37 

XV 


Xvi  LIST    OF    ILLUSTRATIONS. 

KIGVRE  OPPOSITE   PAGE 

39  Choroiditis  showing  vitreous  opacities 41 

40  Choroiditis  causing  flat  detachment    41 

41  Choroiditis,   stage  of  repair 44 

42  Choroiditis,   atrophic  stage    44 

43  Choroiditis,   final  stage  of  atrophy 44 

44  Exudates  in  suprachoroidea 44 

45  Choroiditis,   atrophic  stage   44 

46  Choroiditis,   atrophic  stage   •    44 

47  Choroiditis,   organized  connective  tissue  of  suprachoroi- 

dea      44 

48,  49      Cicatricial  formation  in  choroid 45 

50  Formation  of  fibrous  tissue  in  choroid  of  phthisical  eye.  .  45 

5  1             Formation  of  fibrous  tissue  in  the  choroid 45 

52  Ossification  of  the  choroid 45 

53  Tuberculosis  of  the  choroid 51 

54  Typical  miliary  tubercle    51 

55  Complete  excavation  of  disc,  non-glaucomatous 58 

56,  57      Colloid  degeneration  of  choroid  (Drusen)    58 

58  Senile  degeneration  of  retina 61 

59  Rupture  of  choroid,   extending  into   nerve   sheath    ....  61 

60  Rupture  of  choroid,   extending  into   optic   nerve    61 

61  Sclerotic  changes  in  central  artery  and  vein 67 

62  Retinal  \'ein  dilated  from  pressure  of  sclerosed  artery.  .  67 

63  Obstructive   endophlebitis    68 

64  Nearly  complete  obstruction  of  retinal  artery 68 

65  Concentric  narrowing  of  lumen  of  central  artery 68 

66  Dilation  of  perivascular  sheath  of  sclerosed  vessel 68 

67  Syphilitic  round  cell  infiltration  of  vascular  wall 68 

68  Syphlitic  cicatricial  formation  in  vascular  wall 68 

69  Linear  hemorrhage  in  nerve  fiber  layer  of  retina 71 

70  Hemorrhages  involving  deeper  layers  of  retina 71 

71,  72     Hemorrhages  involving  rods  and  cones    71 

73  Small  hemorrhagic  extravasation  near  macula 71 

74  Angiopathic  albuminuric  retinitis    85 

75  Angiopathic  albuminuric  retinitis,  cystoid  spaces    85 

76  Edematous  infiltration  around  macula,  albuminuric  reti- 

nitis      85 

77  Retinal  discoloration  after  removal  of  hemorrhage.  ...  85 


LIST    OF    ILLUSTRATIONS.  XVU 

FIGURE                                                                                                                                                                          OPPOSITE  PAGE 

78  Deep  hemorrhage,  replaced  by  cicatricial  tissue 85 

79  Granular  body  in  retinal  layers,  albuminuric  retinitis.  .  .  113 

80  Sclerotic  changes  in  choroidal  vessels,  syphilis 113 

81  Nearly  complete  closure   of  central    artery,   endarteritis 

nodosa    113 

82,83  Closure   of  central   artery,   with  congestion  of  choroid.  117 

.    84  Closure  of  central  artery,   longitudinal   section    129 

85  Closure  of  central   artery  by  active  endarteritis 129 

86  Closure  of  central   artery  by  endarteritis,  cell  prolifera- 

tion      130 

87  Complete  closure  of  both  branches  of  central  vein 130 

88  l-'ndarteritic  obstruction,  converted  into  connective  tissue  130 

89  Degenerative  changes,  obscuring  nature  of  obstruction.  .  131 

90  Artefact,  obscuring  nature  of  obstruction 131 

91  Endothelial  proliferation  due  to  traumatism 133 

92  Degenerative  changes  following  obstruction 133 

93  Retina,  two  weeks  after  closure  of  central  ?rtery 133 

94  1  he  retina  after  closure  of  central  artery 136 

95  Field  after  closure  of  inferior  temporal  artery 136 

96  Collateral  branch  at  termination  of  thrombosis 148 

97  Canalization  of  thrombus 148 

98  Another  instance  of  so-called  canalization 148 

99  Central  vein  closed  by  phlebitis  and  thrombosis 149 

100  Sclerosis  of  central  vein   149 

101  \'enous  occlusion  converted  into  connective  tissue   153 

102  (ilaucoma,  secondary  to  venous  thrombosis 153 

103  Section  of  retina  destroyed  by  hemorrhage 163 

104  Closure  of  branch  retinal  vein 163 

105  Typical  detachment  of  the  retina 163 

106  Detachment  of  retina  and  choroid 163 

107,  108  Cystic  formation  in  retinal  detachment 166 

109  Detachment  of  retina    (field)    166 

1  10  Fibers  of  the  vitreous  after  hardening 171 

1  I  1  Space  between  vitreous  and  retina,  artefact 171 

1  12  Shrinkage  of  vitreous  without  retinal  detachment 174 

1  1  3  Spontaneous  detachment  of  the  retina 1  74 

1  14  Detachment  of  retina  due  to  contraction — iridorvclitis.  .  174 

115,  116  Detachment   of  retina  due  to  thrombus  of  vena  vorticos.T  175 


xvili  LIST    OF    ILLUSTRATIONS. 

FIGURE                                                                                                                                                                        OPPOSITE  PAGE 

117  Congenital  detachment  of  the  retina 175 

118  Old,  degenerated  detachment    176 

119  Detachment  showing  connective  tissue  degeneration  ....  176 

120  Retina  two  weeks  after  spontaneous  detachment 176 

121  Ring  scotoma,  retinitis  pigmentosa 180 

122  Retinitis  pigmentosa   (field)    180 

123  Retinitis   pigmentosa,  flat  preparation 187 

124  Retinitis  pigmentosa,  atrophy,  except  in  region  of  macula  187 

125  '    Retinitis  pigmentosa,  degeneration  of  retinal  layers.  ...  188 

126  Retinitis  pigmentosa,  highly  atrophic  choroid 188 

127  Retinitis  pigmentosa,  simulated  by  injury 188 

128  Retinitis  punctata  albescens  (field)    193 

129  Maculocerebral  degeneration    (field)    193 

130  Amaurotic  family  idiocy;  retinal  fold,  art.efact 207 

131  Amaurotic  family  idiocy,  optic  atrophy 207 

132  Commotio  retins,  iris  pressed  against  cornea 207 

133a  Concentric  contraction,  wood  alcohol  poisoning  (field)  .  .  209 

133b  Central  tobacco  scotoma   209 

134,  13  5,  136   Retinitis  proliferans 211 

137,  138   Retinitis  proliferans 212 

139,  140,  141    Choked  disc,  microscopic  sections 220 

142,  143    Choked  disc,   microscopic  sections    221 

144  Choked  disc,    following  Injury  to  crmium 221 

145  Optic  neuritis  in  cerebral  tuberculosis 221 

146  Relation  of  accessory  sinuses  to  optic  nerve 227 

147  Ethmoidal  contraction  of  color  fields 227 

148  Atrophy  in  post-operative  neuritis   (field)    230 

149  Optic  neuritis,  post-operative  (field)    230 

1 50  Tabetic  optic  atrophy  (field)    232 

151  Minimum  contraction,  tabetic  optic  atrophy 232 

152  Optic  atrophy  of  tabes,  late  stage 234 

153  Paracentral  scotomata  in  multiple  sclerosis 234 

154  Oxycephaly,  tower  skull    235 

155  Round  or  normally-shaped  globe 247 

156  Posterior  bulging  of  globe  in  myopia 247 

157  Myopia  with  staphyloma  veriim    248 

1 5  8  High  myopia 248 

159  High  myopia,  disappearance  of  choriocapillaris 251 


LIST    OF    ILLUSIRAIIOXS.  XIX 

FK;URK  opposite  PAGE 

160  High   myopia,  thinning  of  sclera 251 

161  The  normal  filtration  angle 25  1 

162  Glaucoma,   intraocular  hemorrhage    256 

153  Glaucoma    fulminans    256 

164  Glaucoma    (field)    256 

165,  166   Glaucoma  of  long  standing 258 

167  Enlargement  of  ciliary  body  In  advanced  lile 258 

168  Glaucoma,  occlusion  of  the  pupil 261 

169  Iris  hombe   261 

170  Total  posterior  synechia    261 

171  Cvst  of  pars  ciliaris  retinae 261 

172  Secondary  glaucoma,   total  anterior  synechia 261 

173  Secondary  glaucoma,   lens  blocking  filtration  angle   ....  261 

174  Epithelial  cyst  following  corneal  wound 261 

175  Cystic  space  in  obliteration  of  anterior  chamber 261 

176  Glaucoma,   epithelium  blocking  filtration  angle 261 

177  Glaucoma   due  to  spontaneous  dislocation  of  lens 261 

178  Glaucoma   due  to  spontaneous  dislocation  of  lens 262 

179  Glaucoma   following  cataract  extraction 262 

180  Sarcoma  of  choroid,  drawing  iris  Irom  cornea 262 

181  Glaucoma,   due  to   sarcoma  of  choroid    262 

182  Glaucoma,   due  to  cyst  of  iris  and  ciliary  body 262 

183  Glaucoma,   blocking  of  angle  by  cells  from  cornea  and  iris  262 

184  Old  glaucoma,  atrophy  of  iris  and  ciliary  body 263 

185  Ciliary   staphyloma     263 

186  Intercalary  staphyloma 263 

187  Equatorial   staphyloma    264 

188  Early  glaucoma,  pit-like  depressions  on  the  disc 264 

189,  190   Early  glaucomatous  cupping  of  the  disc 264 

191  Glaucomatous  cupping,  later  stage 264 

192  Glaucomatous  excavation  with  new  connective  tissue.  ...  271 

193  Cilaucomatous  excavation  with  new  vascularized  tissue  .  .  271 

194  Cilaucomatous  excavation  with  fibrous  projection   271 

195  Buphthalmus    271 

196  Sarcoma   of  choroid — stage  of  extrabulbar  extension.  .  .  271 

197  Sarcf)ma   of  choroid,  projecting  into  \itreous 271 

198,  199   Sarcoma   of  choroid,   projecting  into  \itre()us 271 

200  I- hit  sarcoma  of  the  choroid 271 


XX  LIST    OF    ILLUSTRATIONS. 

FIGURE  OPPOSITE  PAGE 

201  Alveolar  sarcoma,  endothelioma  of  choroid  .  . 271 

202  Sarcoma  of  the  choroid,  spindle-celled 272 

203  Angio-sarcoma  of  the  choroid 272 

204  Alveolar  sarcoma  of  the  choroid — endothelioma 273 

205,  206  Perithelioma  of  choroid  and  ciliary  body 273 

207  Sarcoma   of  choroid,   intracellular  and  isolated  pigment  274 

208  Sarcoma   of  choroid,  migration  of  cells  along  optic  nerve  274 

209  Small  leucosarcoma  of  the  choroid 274 

210  Sarcoma  of  choroid  with  equatorial  staphyloma    277 

211,  212  Metastatic  carcinoma  of  choroid    277 

213  Glioma   of  the   retina,  gross  specimen 280 

214,  215  Glioma   of  the   retina,  microscopic  specimens    280 

216  Glioma,   ganglion  and  neuroglia  cells    283 

217,218  Metastatic  endophthalmitis,  umbrella-like  detachment..  283 

219  Metastatic  endophthalmitis    • 285 

220  Circumscribed  exudate  due  to  injury,  simulating  tumor.  .  285 

221  Post-lental  membraneous  exudate    285 

222  Sarcoma   of  ciliary  body,  luxating  lens    288 

223  Sarcoma   of  choroid  and  ciliary  body,  poorly  pigmented  288 

224  Conglomerate  tubercle  of  ciliary  body,  invading  lens  ....  288 

225  Conglomerate  tubercle  of  ciliary  body,  perforating  sclera  288 

226  Metastatic  carcinoma  of  ciliary  body 288 

227  Metastatic  hydronephroma  of  ciliary  body 288 

228  Cyst  of  the  pars  ciliaris  retinae 288 

229  Uveal  pigment  simulating  growths  in  ciliary  body 288 

230,  231  Telangiectasis  of  ciliary  body    289 

I'M  Orbital  tumor,  causing  localized  hypermetropia 291 

233  Tumor  of  optic  nerve    291 

234  So-called  glioma  of.  optic  nerve    291 


LIST  OF  COLORED  PLATES. 

OPPOSITE    PAGE 

1  Coloboma  of  the  choroid    20 

2  Miliary  tuberculosis  of  the  choroid 50 

3  Rupture  of  the  choroid 60 

4  Detachment  of  the  retina    162 


PART  II. 

CONTENTS  OF  PORTFOLIO  NO.  I. 

INDEX  CARD. 

DIAGNOSTIC  CARDS. 

1.  Differentiation  of  Coloboma  of  the  Choroid  from  Cicatrix  of  the 
Choroid;  Coloboma  of  the  Optic  Nerve  from  Glaucoma;  Congenital 
Albinism  from  Acquired  Albinism  of  the  Eyeground. 

2.  Differentiation  of  Flxudative  Stage  of  Disseminated  Choroiditis  from 
Albuminuric  Retinitis  and  Diabetic  Retinitis. 

3.  Differentiation  of  Retinal  Opacity  of  Choroiditis  from  Retinal  Opacity 
of  Arterial  Closure;  Chorioretinitis  Pigmentosa  from  Retinitis 
Pigmentosa. 

4.  Differentiation  of  Focal  Tubercular  Choroiditis  from  Exudative 
Choroiditis;  Albuminuric  Retinitis  from  Chronic  Choroiditis. 

5.  Differentiation  of  Simple  Optic  Atrophy  from  Neuritic  Atrophy, 
Retinitic  Atrophv  and  Glaucoma. 

6.  Differentiation  of  Acute  Glaucoma  from  Acute  Iridocyclitis  and 
Iritis. 

7.  Differentiation  of  Sarcoma  of  the  Choroid  from  Metastatic  Carci- 
noma of  the  Choroid. 

8.  Differentiation  of  Glioma  of  the  Retina  from  Pseudoglioma. 

STEREOGRAMS   1-35    (incmsivk). 

1.  Use  of  the  Ophthalmoscope.      (Direct  method.) 

2.  Normal  Fundus  Oculi. 

3.  Retinal  Eight  Reflexes  in  Negroid  Fundus. 

4.  I  he  Retinal  Vessels. 

5.  I^hysiologic  Excavation  of  Optic  Disc. 

6.  Coloboma   of  the   Choroid. 

7.  Coloboma   of  the   Choroid  and  Opti-c  Nerve. 

8.  Coloboma   of  the   Macula. 

9.  Coloboma   of   the  Optic  Nerve  and  Nerve  Sheath. 


XXI 1  ,  CONTENTS. 

10.  Inferior  Optic   Crescent. 

11.  Opaque,  or  Medullated  Xerv^e  Fibers. 

12.  Prepapillary  Retinal  Artery. 

13.  Pseudoneuritis  and   I'ortuous  Vessels  in  Hypermetropia. 

14.  Persistent  Canal  of  Cloquet. 

15.  Albinism  of  the  Eyeground. 

16.  Punctate  Condition  of  the  Fundus  (Gunn's  or  Crick  Dots). 

17.  Choroiditis    Disseminata,   exudative   stage. 

18.  Choroiditis   Disseminata,   exudative   stage. 

19.  Central  Syphilitic  Choroiditis,  exudative  stage. 
20..  Choroiditis  Disseminata,  cicatricial  stage. 

21.  Central  Choroiditis,  commencing  cicatrization. 

22.  Chorioretinitis  Pigmentosa,  Retinitic  Optic  Atrophy. 

23.  Tuberculosis  of  the  Choroid. 

24.  Diffuse  Chorioretinitis,   atrophic  stage. 

25.  Vascular  Degeneration,  or  Sclerosis  of  the  Choroid. 

26.  Atrophy  of  Choroid  causing  Albinism  of  the  Eyeground. 

27.  Senile    Changes    in    the    Fundus;    Colloid    Excrescences     (Drusen), 
Macular  Degeneration  and  Arteriosclerosis. 

28.  Rupture  of  the  Choroid. 

29.  Diffuse  Retinal  Arteriosclerosis,  primary  stage. 
.30.  Advanced  Retinal  Arteriosclerosis. 

31.  Arteriosclerosis   and  Neuritis. 

32.  Arteriosclerosis  with   Hemorrhages. 

33.  Preretinal,  or  Subhyaloid  Hemorrhage. 

34.  Angiopathic  Retinitis,  precursory  stage. 

35.  Albuminuric  Retinitis,   exudative  stage. 


CONTENTS  OF  PORTFOLIO  NO.  II. 

STEREOGRAMS  36-79    (inclusive). 

36.  Albuminuric    Retinitis,    degenerative   stage. 

37.  Albuminuric    Retinitis,    atrophic   stage. 

38.  Diabetic   Retinitis. 

39.  Leukemic  Hemorrhagic   Retinitis. 

40.  Retinitis   Circinata. 

41.  Closure   of  the   Central    Retinal  Artery. 

42.  Closure   of  the   Central   Retinal  Artery. 

43.  Closure   of   Central   Retinal  Artery  with  Visible  Collateral   Circula- 
tion and  (jranular  Blood  Current. 

44.  Retinal  Atrophy  following  Closure  of  Central  Artery. 

45.  Closure  of  Branch  of  Central  Artery. 

46.  Closure,  or  Thrombosis  of  Central  Retinal  Vein. 

47.  Closure   of   Central  Vein  with  Neuritis,  or  Hemorrhagic  Neuroreti- 
nitis. 

4S.  Closure   of  the  Superior  Retinal  \'ein. 

49.  Post-thrombotic  Changes  in  the  Fundus. 

50.  Incipient,  Flat  Detachment  of  the  Retina. 

51.  Well-marked,  Recent  Detachment  of  the  Retina. 

52.  ^)ld,  Translucent  Detachment  of  the  Retina. 

53.  Retinitis    Pigmentosa,   Retinitic  Optic  Atrophy. 

54.  Retinitis   Punctata  Albescens. 

55.  Maculo-cerebral    Degeneration,   advanced   stage. 

56.  .Maculo-cerebral    Degeneration,   early   stage. 

57.  Amaurotic  Family  Idiocy. 

58.  Retina  in  Congenital  Cyanosis,  or  Cyanosis  Retlnje. 

59.  I'raumatic  I'.dema  of  the  Retina,  or  Commotio  Retinae. 

60.  Retinitis   in   Wood  .Alcohol  Toxaemia. 

61.  Retinitis   I'roliferans. 

62.  Retinitis  Striata. 

63.  Papilledema,  or  Choked  Disc  in  Brain    Tumor. 

64.  Pai)illedema,  or  Choked  Disc  in  Brain  Tumor. 

65.  Papilledema  in  Disease  of  the  Accessory  Sinuses. 

66.  Optic  Neuritis  in  Postoperative  Cerebral  Sinus    f  hrombosis. 

67.  Simple,  or  Grey  Optic  Atrophy  in   labes. 


Xxiv  CONTENTS. 

68.  Simple  Optic  Atrophy  in  Multiple  Sclerosis. 

69.  Postneuritic  Optic  Atrophy. 

70.  Non-progressive  Myopia. 

71.  Progressive,  Pernicious,  or  Malignant  Myopia. 

72.  Simple  Glaucoma,  with  Total  Excavation  of  Disc. 

73.  Glaucoma  with  Advanced  Arteriosclerosis,  the  so-called  Hemorrhagic 
Glaucoma. 

74.  Leuco-sarcoma  of  the  Choroid. 

75.  Melano-sarcoma  of  Choroid  with  Total  Detachment  of  Retina. 

76.  Glioma  of  the  Retina. 

77.  Pseudoglioma,  or  Metastatic  Endophthalmitis. 

78.  Sarcoma  of  the  Choroid  and  Ciliary  Body. 

79.  Optic  Atrophy  and  Hypermetropia,  secondary  to  Tumor  of  the  Optic 
Nerve. 


Chapter  T. 
EXAMIXAIIOX  OF  THE  EYE. 

The  Ophthalmoscope.  The  eye  is  distinguished  from  all  other 
organs  of  the  body  in  that  its  tissues  are  transparent.  Here  can  be  observ^ed 
currents  of  Hving  blood  circulating  within  the  vessels,  also  the  secret  proc- 
esses of  disease  and  repair.  Within  the  deep  tissues  of  the  eye  we  may  see 
hemorrhages  and  exudates  appear  and  disappear,  blood  vessels  pass 
through  the  varied  stages  of  degeneration,  and  neoplasms  develop  the  per- 
sistence of  which  will  destroy  life.  It  is  unfortunate  that  these  oren  secrets 
should  be  hidden  from  any  practitioner  of  medicine,  particularly,  as  the 
presence  of  many  insidious  diseases  is  manifest  early  in  the  eye,  and  fre- 
quently the  first  intimation  that  nephritis,  arteriosclerosis  or  grave  disease 
of  the  ner\()us  system  is  present  comes  through  an  ophthalmoscopic  ex- 
aminatic;n. 

To  use  the  ophthalmoscope  successfully  is  a  simple  mechanical  pro- 
cedure the  attendi:it  difficulties  of  which  have  been  overrated.  The  fault 
with  all  beginners  in  ophthalmoscopy  is  haste.  As  a  matter  of  fact,  no 
method  of  physical  diagnosis  is  more  readily  acquired,  provided  each 
simple  man(ru\'re  is  mastered  in  proper  sequence. 

The  ophthalmoscope  is  a  small  m'irror  in  the  center  of  which  is  an 
aperture  through  which  the  observer  look>.  The  mirror  collects  light  from 
a  lamp  or  other  source  of  illumination  and  reflects  it  through  the  pupil,  into 
the  eye  of  the  subject  (Fig.  1  ).  Electric  ophthalmoscopes  are  now  made 
in  ^vhich  the  source  of  light  is  a  small  electric  lamp  attached  in  front  of 
the  mirror.  This  arrangement  removes  the  chief  difficulty  attending  oph- 
thalmoscopic examinations,  /'.  r.,  manipulation  of  the  mirror  so  as  to 
reflect  light  accurately  through  the  pupil  of  the  subject.  The  electric 
ophthalmoscope  illuminates  the  fundus  perfectly  in  a  lighted  room  and  is 
especially  valuable  for  bedside  examinations  (Fig.  2). 

\\'here  both  subject  and  observer  have  normal  refraction,  a  per- 
forated mirror  is  the  only  instrument 'required  to  obtain  a  \iew  of  the 
fundus.  If.  however,  errors  of  refraction  are  present,  a  correcting  glass 
must  be  employed.  The  observer  may  wear  his  own  glass,  but  refractive 
errors  in  the  subject  are  corrected  by  a  series  of  lenses  attacheil  to  the 
ophthalmoscope  in  such  manner  that  the   requisite  lens  is  easily  brought 


2  THE    FUNDUS    OCULI. 

into  the  aperture  of  the  mirror  (  Fig.  3 ) .  The  beginner  in  ophthalmoscopy 
should  employ  as  a  subject  for  examination  a  young  person  whose  pupils 
naturally  are  wide,  or  someone  whose  pupils  have  been  dilated  by  a 
mydriatic,  bearing  in  mind  the  danger  of  using  powerful  mydriatics  on 
those  adults  who  possess  a  predisposition  to  glaucoma. 

For  descriptive  purposes,  the  several  steps  of  an  ophthalmoscopic 
examination  will  be  presented  in  the  following  order:  (1)  Illumination; 
(2)   Direct  examination;  (3)   Indirect  examination. 

Illumination.  The  examination  is  conducted  in  a  dark  room. 
Both  subject  and  observer  are  seated.  A  lamp  with  a  large  flame  is  placed 
near  and  slightly  behind  the  subject's  head,  on  the  same  side  as  the  eye  to 
be  examined.  For  this  purpose  I  prefer  the  round  flame  oil  or  gas  burner. 
The  observer,  facing  the  subject  at  the  distance  of  about  one  foot,  holds 
the  back  of  the  mirror  close  to  his  own  eye,  peeps  through  the  aperture 
and  throws  a  reflection  of  the  lamplight  through  the  pupil  of  the  sub- 
ject fStergm.  1).  The  observer  employs  the  right  eye  to  examine  the 
right  eye  of  the  subject  and  vice  versa.  The  subject  should  not  look 
directly  into  the  mirror,  but  just  past  the  head  of  the  observer.  As  light 
reflected  from  the  mirror  enters  the  eye  of  the  subject,  the  pupil  presents 
a  brilliant  red  illumination,  due  to  rays  from  the  fundus  reflected  into  the 
mirror  and,  incidentally,  through  the  aperture  into  the  eye  of  the  observer. 
No  fundus  details  are  seen  in  simple  illumination  if  the  eye  is  normal. 
Although  this  procedure  is  easily  performed,  it  should  be  thoroughly  mas- 
tered, inasmuch  as  manipulation  of  the  mirror  ii  this  and  other  positions 
is  the  chief  technical  factor  in  ophthalmoscopy.  Having  acquired  sufficient 
dexterity  to  hold  the  fundus  reflex  with  the  mirror,  the  observer  rotates  a 
convex  lens  of  6  D.  into  the  aperture  cf  the  ophthalmoscope  and  ap- 
proaches the  eye  of  the  subject  until  the  red  reflex  is  sharply  outlined  by  the 
edge  of  the  pupil.  To  illuminate  all  parts  of  the  fundus  the  subject  flx.^s  his 
eye  in  various  positions,  /'.  e.,  to  the  right,  to  the  left,  upwards,  downwirds, 
etc.  Simple  illumination  affords  valuable  information.  Thus,  if  the 
media  are  clear  and  the  fundus  normal,  the  pupil  will  be  uniformly  red 
in  all  positions  of  the  eye,  if,  however,  opacities  exist  in  the  cornea, 
aqueous,  lens  or  vitreous,  they  will  obstruct  the  light  and  appear  dark  or 
black.  \'itreous  opacities  usually  appear  as  floating  objects,  while  incipient 
cataract  presents  fixed  opacities,  often  arranged  like  the  snokes  of  a  wheel. 
Gross  hsions  in  t'^e  choroid  and  retina  change  the  color  of  the  fu'-»dus 
reflex.  For  example,  a  detached  retina  gives  a  pearl-grey  reflex,  chr^roidal 
cicatrices  and  retinal  exudates  appear  white,  while  an  intraocuhr  tumor 


ImK.   1. 


riK.  ■■''. 


Fiif.    1      fjjjlitli.ilnioscnix-    ( nhvcrso).       r'ijj:.    ?. —  Electric    O])lith;ilini)<C(ii)0.       Fig.    3- 
Oplitlialmo-  roue   (reverse  I 


i:.\.\Mix.\riox  OF    rnK   eve,  3 

may  give  no  reflex  and  the  pupil  becomes  black.  The  strength  of  the 
convex  lens  in  the  aperture  of  the  ophthalmoscope  should  now  be  increased 
to  12  or  even  18  D.  and  the  eye  closely  approached  until  details  in  the  iris 
are  in  accurate  focus.  With  this  glass  the  finest  opacities  in  the  cornen 
and  lens  may  be  seen.  Whether  a  given  opacity  is  in  front  or  behind  t+ie 
iris  is  determined  by  the  test  of  parallactic  displacement.  This  is  per- 
formed by  noting  the  relative  position  which  the  opacity  bears  to  the 
margin  of  the  pupil.  The  observer  then  slowly  moves  his  head  from 
side  to  side  and  the  opacity  will  appear  to  follow  in  the  same  direction  if 
situateti  behind  the  iris:  on  the  other  hand,  it  will  appear  to  mo\'e  in  a 
direction  opposite  to  that  of  the  head  if  situated  in  front  of  the  iris.  In 
a  like  manner  the  relati\'e  positions  of  any  two  objects  in  the  fundus  may 
be  determined  hv  the  parallactic  test. 

The  obser\-er  next  proceeds  to  examine  details  in  the  eyeground. 
Two  principal  methods  are  employed  to  obtain  an  ophthalmoscopic  view 
of  the  fundus,  /.  c,  the  direct,  in  which  only  the  ophthalmoscope  is  em- 
ployed, and  the  nidirt'ct,  in  which  a  strong  auxiliary  lens  is  interposed 
between  the  ophthalmoscope  and  subject. 

Use  of  the  Ophthalmoscope.  Stereogram  1.  Direct  Method. 
By  the  direct  method  the  obser\'er  looks  into  an  eye  as  through  an  open 
window  and  objects  in  the  fundus  are  seen  erect  in  their  natural  positions. 
If  both  subject  and  observer  have  normal  refraction,  no  lens  is  placed  in 
the  aperture  of  the  ophthalmoscope.  The  lamp  remains,  as  heretofore,  at 
the  side  of  the  eve  to  be  examined.  The  mirror  is  tilted  on  its  vertical 
axis  toward  the  lamp  so  as  to  reflect  light  into  the  pupil.  In  the  electric 
ophthalmoscope  the  lamp  and  mirror  are  fixed  to  the  instrument  in  their 
proper  jjositions.  The  obser\er  comes  \-ery  close  to  the  subject,  keeping 
the  pupil  illuminated,  and  it  the  mirrf)r  is  properly  held  a  distinct  view 
of  the  eveground  will  be  obtained.  If  the  subject  has  small  pupils,  they 
should  be  dilated  with  a  weak  mydriatic,  such  as  cocaine,  euphthalmine, 
homatropine,  etc.  Atropine  should  not  be  employed  tor  this  purpose,  as 
its  prolonged  action  may  precipitate  glaucoma  in  cKlerlv  people  if  a  pre- 
disposition to  this  disease  exists. 

The  following  rules  should  be  obserxed  in  tlirect  examination  of  the 
fundus : 

(  1  )  In  the  primary  position,  the  subject's  eye  should  be  directed 
slightly  to  the  nasal  side,  which  will  bring  the  optic  disc  m  direct  line  of 
the  obser\-cr's  vision. 

(2)    The  observer  should  keep  his  head  jierpendicular  with   that  of 


4  THK     FUNDUS    OCULI. 

the  subject.  If  held  transversely,  the  forehead  will  come  before  the  sub- 
ject's disengaged  eye  which  then,  having  lost  its  point  of  fixation,  will 
move. 

(3)  The  reflection  from  the  ophthalmoscopic  mirror  casts  a  smill, 
central  shadow,  due  to  the  aperture.  Guide  the  light  into  the  subject's  eye 
by  keeping  this  shadow  directly  on  the  pupil. 

(4)  "Fhe  observer  must  relax  his  own  accommodation.  This  is  ac- 
complished bv  looking  at  the  fundus  picture  as  though  it  were  situated 
at  infinite  distance.  If  sought  for  close  at  hand,  the  observer  exercises 
accommodation  and  the  picture  will  be  blurred.  Beginners  invariably 
violate  this  rule. 

(5)  Hold  the  mirror  close  to  the  subject's  eye,  as  in  this  way  a 
larger  field  is  obtained. 

By  the  direct  method  of  examination,  objects  in  the  fundus  are  'Tia9:- 
nified  about  15  diameters  by  the  dioptric  system  of  the  eye.  This  is  i  much 
greater  magnification  than  can  be  obtained  by  the  indirect  method.  The 
direct  method  is  employed  to  study  vascular  changes,  location  and  nature 
of  exudates,  measurement  of  levels  and  all  minutiae  in  the  fundus. 

By  the  direct  method  of  examination,  elevations  and  depressions  in 
the  fundus  are  recognized  and  accurately  measured.  For  example,  a 
swollen  nerve  or  detachment  of  the  retina  has  a  more  hypermetropic  re- 
fraction than  other  parts  of  the  fundus  and  requires  the  addition  of  a 
convex  lens  to  the  ophthalmoscope  to  render  It  distinct.  The  strength 
of  the  lens  necessary  to  accomplish  this  measures  the  height  of  the  eleva- 
tion, the  rule  being  that  3  dioptres  of  refraction  is  equal  to  a  difference 
in  levels  of  1mm.  The  depth  of  a  depression  in  the  fundus,  as  the  floor 
of  a  glaucomatous  cup,  is  measured  in  the  same  manner,  except  that  in 
this  case  a  concave  lens  is  added  to  the  combination  suitable  for  other 
parts  of  the  fundus. 

Indirixt  Method  (Inverted  image).  In  examining  the  eye- 
ground  by  the  iiulircct  method,  the  source  of  light  should  De  slightly  above 
the  subject's  head.  The  observer,  seated  about  18  inches  in  front  of  the 
subject,  holds  a  biconvex  lens  of  14  to  16  I),  at  its  focal  length  before 
the  eye  to  be  examined,  at  the  same  time,  illuminating  the  eye  with  the 
ophthalmoscope  (Fig.  4).  When  properly  held,  the  biconvex  lens  will 
intercept  the  rays  of  light  returning  from  the  subject's  eyeground  and 
will  form  an  inverted,  aerial  image  of  the  fundus  between  the  lens  and  the 
ophthalmoscope.  It  is  more  difl^cult  to  obtain  a  fundus  picture  by  the 
mdirect  than  by  the  direct  method,  but  after  a  little  practice  the  image 


'fe^^#r: '  --d 


Fig.  4. 

Indirect   Method   nf  Oplithalmosonpic   Examination. 


Oblique  Focal  Illumination. 


liXAMlNAllON    OF     llli:    liYE.  -> 

suddenly  will  be  perceived  and  the  observer  is  amazed  that  it  was  not 
seen  before.     The  observer  should  hold  the  lens  between  the  forefinger 
and  thumb  while  the  little  finger  rests  on  the  subject's  temple.     The  plane 
of   the   lens   should   correspond   with   the    plane   of   the    subject's   cornea, 
although  if  given  a  slight  obliquity  certain  annoying  surface  reflexes  will 
disappear.     It  is  desirable  to  hold  the  lens  in  the  left  hand  when  examining 
the  right  eye,  and  in  the  right  hand  when  examining  the  left  eye.       I  he 
image  may  be  obtained  with  the  open  aperture.     It  is  advisable,  however, 
to  look  through  a  lens  of  4  or  5  D.  which  will  relie\e  the  accommodation 
of  the  observer  and  enable  him  to  come  closer  to  the  image.     The  observer 
should  Hrst  find  the  optic  disc,   and  starting  from  this  point  explore  the 
eyeground  in  all  directions.     In  the  indirect  image  the  position  of  objects 
is  reversed;  thus,  the  upper  part  of  the  picture  is  the  lower  part  of  the 
fundus,  the  macula  appears  on  the  nasal  side,  etc.     By  the  indirect  method 
a  magnification  of  but  three  or  four  diameters  is  obtained  as  compared 
with    fifteen    diameters   by   the    direct   method.      Therefore,    the    indirect 
method  is  not  the  best  for  examining  details;  on  the  other  hand,  it  possesses 
the    following   advantages   over   the   direct:     {a)    a   much   larger   field   is 
covered  and  a  more  comprehensi\e  v\2\v  of  the  eyeground  obtained;   (h) 
the   illumination   is   much  brighter   and   the    fundus   can   be   seen   through 
opacities  which  would  obstruct  the  view  by  the  direct  method;    (c)    it  is 
the  only  method  bv  which  a  satisfactory  view  of  the  fundus  can  be  obtained 
in  cases  of  high  mvopia  and  astigmatism;     (^)    a  fundus  picture  can  be 
obtained  through   a   narrow  pupil;    {c)    patients  can   easily  be   examined 
in  the  recumbent  position. 

Focal  (jr  ORi.iQrn  Iiumix atiox.  FAcry  ophthalmoscopic  exami- 
nation should  be  preceded  by  ohl'ujitc  illnni'nial'u))!  of  the  eye.  For  this 
purpose,  the  source  of  light  shouUl  be  situated  a  few  feet  distant  from  the 
eye  to  be  examined.  With  a  convex  lens  of  15  to  20  D.  the  light  from  a 
lamp  or  window  is  concentrated  so  that  the  focal  point  falls  obliquely 
across  the  cornea  (Fig.  5).  As  the  point  ot  light  is  moved  o\er  the 
surface  of  the  cornea,  the  most  tlelicate  lesions  will  be  revealed.  The  iris 
and  lens  may  be  examined  by  this  method  and  e\  en  growths  situated  lar 
forward  in  the  vitreous  may  be  seen.  The  obserxer  may  wear  a  seconti 
con\cx  lens  to  magnify  objects  which  are  illuminated  by  the  oblicjue  method. 

In  diagnosticating  diseases  of  the  fundus  oculi,  the  ophthalmoscopic 
examination  should  be  supplemented  by  all  known  methods  of  detecting 
disease,  particularly  modern  laboratory  tests  including  those  for  syphilis 
and  tuberculosis.     In  this  work,  however,  it  is  appropriate  to  refer  only 


6  riiK  FixDrs  oculi. 

to  those  methods  which  lie  within  the  provi'ice  of  the  ophthilmologist  and 
neurologist,  i.  c,  examination  of  the  visual  Held  and  transillumination  of 
the  eye. 

The  visual  field  is  the  area  in  space  in  which  objects  are  perceived  at 
one  time.  Accurate  vision  for  detail  is  limited  to  a  small  point  in  the 
visual  Held  which  corresponds  to  the  fovea  centralis  (central  or  direct 
vision).  Outside  this  small  area  objects  are  seen  with  diminishing  dis- 
tinctness from  the  center  to  the  periphery  (peripheral  or  indirect  vision). 
Examination  of  the  visual  field  includes  tests  for  color  perception  as  well 
as  for  light.  1  he  power  to  distinguish  colors  varies  in  different  parts  of 
the  retina,  thus,  colors  are  recognized  throughout  the  central  region,  but 
not  at  the  periphery.  Each  color  has  its  own  normal  limitations  in  the 
visual  field,  variations  from  which  are  pathologic.  The  normal  field  for 
white  and  for  colors  is  shown  in  Fig.  6.  Rays  of  light  from  an  object 
cross  each  other  before  reaching  the  retina,  consequently,  the  nasal  field 
corresponds  to  the  temporal  side  of  the  retina  and  vice  versa.  The  normal 
eve  contains  a  blind  spot  (fig.  6)  produced  by  entrance  of  the  optic  nerve. 
Examination  of  the  visual  Held  can  roughlv  be  made  as  follows:  Subject 
and  examiner  should  be  about  two  feet  apart.  If,  for  example,  the  right 
eye  is  under  examination,  the  subject  closes  the  left  eve  and  the  examiner 
closes  the  right  eye.  The  subject  looks  steadily  into  the  eye  of  the 
examiner  while  the  latter  advances  his  hand,  or  other  object,  from  periph- 
ery to  center  of  field,  keeping  it  equiciistant  from  himself  and  subject. 
The  subject  indicates  when  the  object  hand  is  first  perceived,  and  if  the 
examiner  sees  it  at  about  the  same  time,  it  may  be  assumed  that  visual 
perception  exists  in  that  part  of  the  field.  The  color  sense  may  be 
examined  in  the  same  manner  by  suitable  color  cards.  These  methods 
answer  the  purpose  for  bedside  examination  or  as  a  primitive  test  for 
gross  defects,  but  accurate  examination  requires  the  use  of  a  special  in- 
strument known  as  the  perimeter  (Eig.  7)  or  a  tangent  plane  chart 
fl'ig.  8).  The  perimeter  consists  of  a  semicircular  arc  of  black  metal 
which  revolves  upon  its  center  so  as  to  describe  the  figure  of  a  hollow 
sphere.  A  scale  of  concentric  circles  is  marked  on  the  arc.  The  subject 
IS  so  placed  that  the  eye  to  be  examined  is  in  the  center  of  the  hollow 
sphere.  The  eye  is  then  fixed  on  the  center  of  the  arc  while  a  small 
white  or  colored  test  object  is  moved  along  its  inner  surface  from 
periphery  to  center  and  the  number  of  the  concentric  circle  where  the 
test  object  first  comes  into  view  is  recorded  on  a  chart.  By  revolving 
the   arc  into  all   meridians  the  entire  visual   field  may  be   plotted.      For 


•p»fj 


Fig-.  6 — Normal  Visual  Field 
for  White  and  Colors,  a — Nasal 
field  corresponds  to  temporal 
side  of  retina.  b  —  Temporal 
field  corresponds  to  nasal  side 
of  retina. 


Fitf.     7    —    Skeel's 
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Fig.  8. 
Duane's  Tangent  Plane  Chart. 


Fig.  a 


.-ft}' 


Q>  vtiM^i-fr^-ot  >\WwaUm»|i4wu 


Fig.  10. 


'7V     ' 


vr 


f0  to  eo  iti  49  3ie  ?» 


so  df   ip    *>  ill;*    V   *o  Mf_  ^fe_cA     fp,J^Jf>  ^  ^  V- 


^xM^ 


.^" 


Etna  sal    Htmiunopsitt,. 


Fig.  11. 


EXAMIXATIOX    OF    THK    KYK.  / 

mapping  out  small  central  or  paracentral  scotomata  a  tangent  plane  (Fig. 
8)   is  better  than  the  perimeter. 

A  detect  in  the  visual  field  is  known  as  a  scotoma.  Scotomata 
are  classified  according  to  their  situation  as  central,  pericentral,  para- 
central, equatorial  and  peripheral,  also  according  to  form  as,  irregular, 
annular,  sector,  crescentic,  etc.  Disappearance  of  the  field  from  periphery 
toward  the  center  is  called  coucoitr'u  coiitrdction  (Fig.  150).  When 
one  halt  of  the  field  is  destroyed  the  patient  is  said  to  haxe  hemianopia 
(hemianopsia).  The  different  forms  are :  (a)  Homonymous  hemianopia 
in  which  vision  is  lost  on  the  same  side,  right  or  left,  in  each  eye  (Fig. 
9):  (h)  Bitemporal  hemianopia  afiects  both  temporal  fields  (Pig.  10); 
(r)  Binasal  hemianopia  both  nasal  fields  (Fig.  11);  (^/)  Horizontal  or 
latitudinal  hemianopia  is  the  condition  in  which  both  upper  or  lower  fields 
are  destroyed  (Fig.  12).  A  scotoma  is  said  to  be  positive  when  it  is 
perceived  by  the  patient  as  a  black  spot  or  as  a  cloud  in  the  field  of 
vision.  A  neqative  scotoma  is  a  blind  spot  in  the  field  which  is  not  seen 
by  the  patient. 

Tran'SILII'MIN  \TI()\.  (  Diaphanoscopy. )  The  interior  of  the  eve 
may  be  illuminated,  not  only  through  the  pu|)il  but  also  through  the 
sclera,  a  fact  which  has  been  utilized  for  diagnostic  purv^oscs.  Special 
lamps  ha\e  been  devised  by  Leber,  Sachs  and  Wurdemann  which,  when 
placed  against  the  sclera,  behind  the  plane  of  the  iris,  in  a  dark  room,  will 
light  up  the  interior  of  a  normal  eve  and  r'Mider  the  pupil  luminom 
(Fig.  13).  If,  however,  an  opaque  object  lies  between  the  lamp  and 
the  pupil,  it  will  cast  a  shadow  and  the  pupillary  illumination  will  be 
darkened.  For  example,  if  the  *"ransilluminator  is  pre:;sed  against  the 
sclera  at  a  point  corresponding  to  the  attachment  of  a  melanosarcoma, 
the  light  cannot  penetrate  the  tumor  and  the  pupil  remains  black.  If 
the  transilluminator  is  then  mo\  ed  to  a  i")oint  not  occupiei,!  by  the  sarcoma, 
the  pupil  becomes  illuminated.  Anorhcr  lorni  of  transilluminator,  known 
as  the  diaphanoscope,  is  jilacetl  in  the  mouth  and  pharynx.  In  this 
position  the  light  is  diffused  through  the  batk  of  the  eye,  thus  re\ealing 
o});i(iue  objects  situated  posteru)r  to  the  equator.  iransilluiiuiiariou  is 
the  most  reliable  test  we  possess  for  diagnostiinring  sarcoma  of  the  cho- 
roid. It  may  fail,  however,  under  the  following  conditions:  (  1  )  When 
the  aperture  in  the  lamp  is  not  entirely  coxered  h\'  the  tumor;  (2)  A 
small  leucosarcoma  may  cast  little  shadow,  in  such  case,  if  the  illumina- 
tion is  reduced  a  shadow  may  be  demonstratetl ;   {^)    In  diffuse  sarcoma; 


8  THE    FUNDUS    OCULI. 

(4)  When  the  base  of  a  sarcoma  is  attached  to  the  posterior  pole  and 
the  head  forms  an  elongated  tumor  directed  forwards  (Fig.  14*).  In 
transillumination  the  intensity  of  the  light  should  be  varied,  a  strong 
Hght  being  required  when  the  globe  is  filled  with  blood  or  contains  a 
dense  tumor,  while  a  dim  light  will  be  nece3sary  to  demonstrate  sli'^ht 
variations  in  the  luminosity  of  the  pupil.  The  following  is  formulated 
from  Friedenberg's  paper  on  transillumination : 

In  normal  eyes  the  sclera  always  is  translucent  and  the  transscleral 
glow  illuminates  the  pupil. 

Corneal  opacities,  however  dense,  offer  no  obstruction  to  the  light. 

Recent,  dense  pannus  slightly  dims  the  glow. 

Uveal  pigment  in  the  cornea  and  elsewhere  is  an  absolute  barrier 
to  the  light. 

No  form  of  cataract  offers  the  slightest  obstruction  to  light  in 
transillummation. 

In  siderosis  of  the  lens  the  light  is  slightly  dimmed. 

The  choroidal  pigment  is  so  scattered  that  it  offers  no  obstruction  to 
transillumination;  on  the  other  hand,  the  closely  packed  pigment  of  the 
ciliary  body  and  iris  is  an  absolute  barrier  to  light  and  the  normal  iris 
is  opaque  regardless  of  its  color.  In  atrophy  of  the  iris  and  in  albinos, 
conditions  in  which  pigment  is  absent,  the  light  readily  passes  through  the 
iris  stroma.  Secondary  membranous  cataract  obstructs  light  only  when 
It  contains  a  blood  clot  or  uveal  pigment.  Freshly  extravasated  blood 
slightly  dims  the  light  but  clotted  blood  entirely  obstructs  it.  Simple  de- 
tachment of  the  retina  is  absolutely  transparent.  Melanosarcoma  of  the 
choroid  casts  a  dense  shadow  which  dims  the  pupil  and  darkens  the  sclera  at 
the  site  of  attachment.  Glioma  of  the  retina  casts  no  shadow  unless  it  has 
undergone  calcareous  degeneration. f  By  combined  use  of  the  ophthalmo- 
scope and   transilluminator   the   visible   area    of   the    fundus   is   enlarged. 

Puncture  is  sometimes  employed  to  determine  the  solid  or  fluid 
character  of  retinal  elevations.  If  an  aspirating  needle  is  employed  it  may 
enter  a  tumor  and  withdraw  blood  and  tumor  cells.  Frequently,  however, 
the  needle  has  failed  to  encounter  a  growth  when  one  was  present.     This 

^■' From   X.  Y.  Post  Graduate  Hospital.     Service  of  Dr.   .\.   K.   Da\is. 

t  Glioma  of  the  retina  casts  little  or  no  shadow  in  tranMlluniination  because,  (1) 
glioma  is  a  retinal  growth,  consequently,  a  space  exists  between  tumor  and  sclera  througli 
which  light  enters  the  eye;  (2)  glioma  contains  no  pigment. 


"ffrAv/^irwtjuJt.  mwvi<M^(r'»Attt' 


FiK.  iii. 


mi¥ 


Fig.  r^. 
Wiirdemann's  Transilluminatnr. 


liic.  14. 
Sarcoma  of  the  Choroid  so  situated  that  Transillumination  may  not  estaijlish  diagnosis. 


KXAMIN  AIIOX    OF     llll.     l.VK. 


method  of  diagnosis  is  seldom  employed,  as  there  is  a  well  founded  belief 
that  the  point  of  puncture  affords  ready  egress  to  tumor  elements. 

Intkaoci'LAR  Prissure.  In  every  examination  of  the  eye  the  in- 
traocular pressure  should  be  estimated  as  a  matter  of  routine. 

The  eye  is  a  soft  capsule  filled  with  Huids  under  a  pressure  of  from 
20  to  30  mm.  Hg.  (  1 8  to  23  mm.  Hg.  according  to  Schiotz ).  I'his 
normal  pressure  produces  the  icus'ion  of  the  ocular  walls  and  maintains  the 
form  of  the  globe.  Any  marked  variation  from  this  standard  is  pathologic. 
Thus,  in  glaucoma  pressure  may  rise  to  more  than  100  mm.  Hg.  or  it 
may  fall,  as  in  phthisis  bulbi,  to  zero.  Intraocular  pressure  is  roughly 
estimated  bv  the  sense  of  touch,  and  with  accuracy  by  means  of  instru- 
ments. The  tension  of  the  walls  varies  with  intraocular  pressure  and 
this  may  be  estimated  by  palpating  the  eye  with  the  fingers  (digital 
tonometry).  If  this  simple  method  is  practiced  by  physicians  on  healthy 
eyes  thev  soon  acquire  the  Idcliis  cniditiis  which  enables  them  readily  to 
distinguish  any  marked  \ariatIons  from  the  normal  tension.  The  follow- 
ing symbols  are  employed  to  record  the  examiner's  impression  as  to  the 
tension  of  a  particular  eve:  T  is  emploved  to  indicate  tension;  Tn,  --^ 
normal  tension.  I'he  degrees  of  increased  tension  (hypertony)  are 
indicated  by  T  -\-  1,  noticeably  increased;  T  -f-  2,  greatly  increased; 
T  +  3,  Stone-like  hardness.  In  a  like  manner  diminished  tension  ( hy- 
potonv)  is  designated  bv  the  degrees  of  softness  as  T —  1;  I  — 2; 
T  — .3. 

Dkjii'AI.  ToxoMi/rKV  is  performed  by  palpating  the  eye.  The  pa- 
tient is  directed  to  look  slightly  downward  so  as  to  get  the  cornea  out  of 
the  way;  then,  the  tips  of  the  two  index  fingers  are  gently  placed  upon  the 
globe  through  the  closed  upper  lid  and  alternately  pressed  against  the 
eye  exactly  as  in  palpating  an  abscess  or  tumor  for  fluctuation  (Fig. 
15).  1  he  eye  should  not  be  directed  very  far  below  the  horizon, 
otherwise  pressure  of  the  extrinsic  muscles  will  increase  tension.  It  is 
desirable  to  take  tension  behind  the  cornea  near  the  equator,  to  accomjilish 
which,  the  back  of  the  examiner's  fingers  should  just  touch  the  margin 
of  the  orbit.  In  palpating  the  eve  the  fingers  will  be  steadied  it  they 
barely  brush  each  other,  judgment  as  to  \ariations  in  the  tension  of 
an  eve  should  be  controlled  by  comparison  with  the  fellow  eye  or  with 
the  normal  eye  of  another  person.  The  normal  eve  ilimples  under  the 
fingers  while  m  absolute  glaucotiia  it  ma\'  leel  hard  as  stone. 

After  instilling  a  solution  of  holocainc  and  tlirecting  the  patient  to 
lor)k    upw.ird.    tension    can    be   tested    by    apph'ing   the    fitigers    directly   to 


10  THE    FUNDUS    OCULI. 

the  sclera  below  the  cornea.  This  method  is  employed  when  the  lids  are 
swollen  or  the  conjunctiva  chemotic;  under  any  conditions  it  is  the  most 
accurate   method   ot"   employing   digital    tonometry. 

Instrumental  Tonometry  is  performed  in  two  ways: 
(  1 )    By  the  manometer,   an  instrument  which  connects  the   interior 
of  the   eye  directly  with  the  mercurial   column   through   a   canula   which 
perforates  the  sclera.     Obviously,  this  method,   which  alone  is  accurate, 
cannot  be  employed  clinically. 

(2)  By  the  tonometer,  an  instrument  by  which  intraocular  pressure 
is  estimated  from  the  resistance  which  the  walls  ofter  to  external  in- 
dentation. Among  the  various  instruments  designed  for  this  purpose 
only  that  of  H.  Schiotz  has  come  into  general  use  (Fig.  16).  This 
instrument  is  applied  to  the  cornea  after  the  latter  has  been  anesthetized 
with  a  2  %  solution  of  holocaine  which  does  not  dilate  the  pupil.  It  is  a 
reliable  index  of  intraocular  pressure  and  should  be  employed  in  all 
doubtful  cases. 


Fig.   15. 

Digital  Tonometry. 


fP-' 

1-iK    JO. 

Schiotz  Tonometer. 


Chaptkk  TI. 
THE   NORMAL  FUNDUS. 

Stereogram  2.  Blond  flxdis.  Right  eyeground  of  a  healthy, 
light  complexioned,  Swedish  girl.  The  fundus  color  is  a  bright  yellowish 
red,  indicating  that  pigment  is  scanty  in  both  retinal  epithelium  and 
choroid.  In  the  macular  region  and  aroimd  the  nerve  the  fundus  darkens. 
The  macula  exhibits  a  visible  foveal  reHex.  The  choroidal  vessels  are 
indistinctly  outlined  but  may  be  recognized  as  red,  anastomosing  bands 
darker  than  the  interxascular  spaces.  No  vessels  are  seen  at  the  macula. 
The  optic  disc  is  nearly  circular  in  form  and  of  normal  color.  Both 
choroidal  and  scleral  ring  are  well  defmed.  The  retinal  vessels  are 
distributed  in  the  most  conventional  manner.  A  small  cilioretinal  artery 
emerges  from  the  temporal  side  of  disc  and  is  directed  toward  the 
macula. 

The  fundus  oculi  comprises  that  portion  of  the  eyeground  which  can 
be  seen  with  the  ophthalmoscope.  In  the  normal  eye  this  includes  a 
little  more  than  the  posterior  hemisphere.  The  ophthalmoscopic  picture  of 
the  fundus  is  created  by  light  reflected  from  the  coats  of  the  eyeball.  7  he 
chief  factor  in  its  production  is  the  choroid  and,  to  a  less  extent,  the 
sclera  and  retina. 

Sclera.  The  sclera  is  composed  of  white  iibrous  connective  tissue 
which,  though  obscured  by  the  red  choroid,  furnishes  a  brilliant  white 
background  which  lightens  the  color  of  the  entire  fundus. 

SupRACHOROiDEA.  The  Sclera  is  connected  with  the  choroid  by  the 
suprachoroidea  or  lamina  fusca,  a  loose  membranous  tissue  arranged  to  act 
as  lymph  spaces.  In  disease  these  spaces  may  be  enormously  distended 
by  exudates  or  blood.  The  outer  layer  of  the  lamina  fusca  contains 
considerable  pigment,  some  of  which  usually  remains  attached  to  the 
sclera  after  the  choroid  has  been  destroyed  by  disease.  The  supra- 
choroidea exerts  little  influence  upon  the  ophthalmoscopic  picture  except 
when  distended  by  pathologic  effusions  (Fig.  44). 

Choroid.  The  brilliant  red  coloring  of  the  Caucasian  eyeground  is 
produced  by  the  choroid.  The  choroid  Is  the  vascular  coat  of  the  eye. 
The  blood  vessels  are  arranged  in  a  manner  peculiar  to  this  membrane 

11 


12  THE    FUNDUS    OCULl. 

in  three  superimposed  layers  (Fig.  17).  The  external  layer  consists  of 
the  larger  vessels,  mostly  veins,  which  anastomose  freely.  The  vessels 
are  united  bv  a  delicate  stroma  containing  pigmented  cells,  called  chro- 
matophores.  1  he  middle  layer  is  made  up  of  medium  sized  vessels.  The 
stroma  contains  but  few  pigmented  cells.  The  tuner  layer,  termed  the 
cliorwcapillaris,  consists  entirely  of  closely  arranged,  wide  capillaries. 
Pigmented  -cells  never  appear  in  the  choriocapillaris  under  normal  con- 
ditions. On  its  inner  surface,  the  choroid  is  separated  from  the  pigmented 
epithelial  layer  of  the  retina  by  a  homogeneous,  elastic  membrane;  the 
lamina  vitrea.  On  the  inner  surface  of  the  lamina  vitrea  the  retinal 
epithelium  forms  a  close  mosaic  of  pigmented,  polygonal  cells  (Fig.  18). 
This  so-called  "  retinal  "  epithelium  develops  from  the  same  blastodermic 
layer  as  the  retina,  but  anatomically  and  pathologically  it  is  a  part  of 
the  choroid.  The  color  and  brilliancv  of  the  ophthalmoscopic  image  is 
modified  by  the  relative  amount  of  pigment  contained  in  the  retinal 
epithelium  and  chromatophores  of  the  choroid.  Usually,  the  amount  of 
pigment  in  the  epithelial  layer  corresponds  to  that  in  the  choroid,  but 
disproportionate  pigmentation  of  the  two  membranes  is  not  uncommon. 
As  a  rule,  the  fundus  ol  a  blonci  individual  is  light  in  color  and  of  a 
brunette  dark,  but  exceptions  are  frequent.  The  following  normal  varia- 
tions in  fundus  pigmentation  produce  the  more  familiar  types  of  eye- 
grounds. 

( 1 )  Excessive  pigmentation  of  both  choroidal  cells  and  retinal  epi- 
thelium, as  in  negroes,  produces  a  poorly  illuminated  fundus,  slate  colored 
at  the  posterior  pole  and  chocolate  color  at  the  periphery  (Strgm.  3). 

(2)  A  moderate  amount  of  pigment  in  the  retinal  epithelium  is 
sufficient  to  hide  the  choroidal  vessels  and  still  permit  the  passage  of  a 
brilliant  reflex  from  the  choroid.  In  such  eyes  the  fundus  has  a  uniform, 
rich  red  color  (Strgms.  16-29-33-58). 

(3)  If  the  retinal  epithelium  is  well  pigmented  and  the  choroid  poor 
in  pigment,  the  epithelium  becomes  visible  and  the  fundus  appears  darkly 
stippled  or  "peppered"   (Strgms.  39-67). 

(4)  If  the  retinal  epithelium  is  poor  in  pigment,  the  choroid  can  be 
seen.  In  such  case,  if  pigment  is  abundant  in  the  intervascular  spaces  of 
the  choroid,  a  tessellated  or  tigered  fundus  is  produced  and  the  choroidal 
vessels  are  of  lighter  color  than  the  background  (Strgm.  55  ) . 

(5)  If  both  epithelium  and  choroid  are  poor  in  pigment,  the  cho- 
roidal vessels  are  darker  than  the  background  and  the  fundus  appears 
reticulated   (Strgms.  2-19-56). 


Fig.    17. 

The  Choroid. 

V    (a)    external   layer,    (b)    midfllc   layer,    (c)    internal   layer 


'\ 


Fig.  18. 
The   Retinal   Epithelium. 


Fig.  19. 
The  Layers  of  the  Retina. 

(a)    retina,    (b)    choroid,   (c)    sclera. 


Fig.  20. 

The   Retina  at  the   Macula. 

Note  thickness  of  layer  of  ganglion  nerve  cells,  a  and  a. 


THE    NORMAL    FUNDUS.  13 

(6)  In  albinos  and  cases  of  fundus  depigmentation,  the  fundus  con- 
tains no  pigment  and  the  choroidal  vessels  are  distinctly  seen  sharply 
defined  against  a  yellowish-white  sclera  fStrgms.  15-26). 

Vessels  of  the  choroid  are  easily  distinguished  from  those  of  the 
retina  by  the  following  peculiarities: 

(a)  They  increase  in  distinctness  and  do  not  narrow  toward  the 
periphery. 

(/;)     They  are  larger  and  more  numerous  than  retinal  vessels. 

(c)  Their  courses  are  erratic.  Groups  of  xessels  frequently  run 
parallel. 

(^)     They  anastomose  with  each  other. 

(c)  Choroidal  vessels  contain  no  central  light  streak  and  arteries 
arc  indistinguishable  from  veins. 

Retina.  The  retina  is  the  inner  or,  as  viewed  with  the  ophthalmo- 
scope, most  superficial  coat  of  the  eve.  It  is  a  transparent  membrane 
contributing  little  toward  the  normal  coloring  of  the  eyeground.  The 
retina  simplv  lies  upon  the  pigmented  epithelium,  its  only  attachments 
being  around  the  optic  nerve,  and  at  its  anterior  border  where  it  joins  the 
choroid  by  a  jagged  line  called  the  ora  scrrata.  Ten  layers  are  recognized 
in  the  retina,  of  which  the  five  outer  are  termed  neuroepithelial  livers 
and  the  live  inner  cerebral  layers  (Fig.  19).  The  nerve  elements  of  the 
retina  are  supported  by  a  connective  tissue  framework  of  ectodermic 
origin.  The  principal  members  of  this  framework  are  the  fibers  of  Miiller 
which  extend  through  the  retina  from  the  internal  limiting  membrane  to 
the  layer  of  rods  and  cones,  where  tliey  expand  a-ul  form  the  external 
limiting  membrane.  "Fhe  macula  hitca,  the  center  of  the  fundus,  is  situated 
about  two  disc  diameters  to  the  temporal  side  of  and  slightly  below  the 
optic  nerve.  This  most  highly  specialized  portion  of  the  visual  organ  is 
the  region  of  acute  vision.  The  macula  is  a  round  or  horizontally  oval 
depression,  about  2  mm.  in  diameter,  in  the  surface  of  the  retina.  The 
macula  is  formed  by  the  following  modifications  in  the  structure  of  the 
retina.  As  the  retina  approaches  the  border  of  the  macula  the  layer  of 
ganglion  cells  increases  in  thickness  from  a  single-celled  layer  to  a  layer 
eight  or  more  cells  deep  (  Fig.  20).  Within  the  macula,  however,  all  layers 
of  the  retma  fuse  and  decrease  in  thickness  until  at  the  fovea  centralis,  or 
center  of  the  macula,  the  membrane  consists  of  little  more  than  a  layer  of 
cones  and  nuclei. 

For  descriptive  purposes  the  eyeball  is  compared  to  the  earth:  thus, 
it  is  said  to  possess  an   anterior  and  a  posterior  pole  between  which   is 


14  THE    FUNDUS    OCULI. 

situated  the  equator.  It  is  further  divided  by  meridians,  as  the  horizontal 
and  the  vertical  meridians.  In  comformity  with  this  method  of  orien- 
tation the  fundus  is  separated  into  ill-defined  zones,  /'.  e.,  a  posterior  polar 
zone,  a  posterior  median  zone  and  an  equatorial  zone,  etc. 

Stereogram  3.  Negroid  Fundus.  Retinal  Light  Reflexes. 
Left  eveground  of  a  healthy  mulatto  boy,  9  years  of  age.  The  color 
of  the  fundus  is  characteristic  of  the  dark-skinned  races  and  is  due  to  an 
excess  of  pigment  in  the  retinal  epithelium  and  choroid  which  obscures 
the  choroidal  reflex.  The  optic  disc  and  retinal  vessels  are  normal.  This 
fundus  is  remarkable  for  the  number  and  brilliancy  of  the  light  reflexes 
which  play  over  its  surface  and  along  the  vessels.  Bright,  silvery  rays 
radiate  in  a  circle  from  the  optic  disc  corresponding  in  their  courses  to 
those  of  the  ner\  e  fibers.  The  edge  of  the  foveal  cup  is  marked  by  a 
semicircle  of  light  which  changes  its  position  with  every  movement  of  the 
mirror. 

Althoufh  the  r::tina  "is  a  transparent  membrane,  usually  invisible,  it 
will  reflect  light,  especially  if  surface  inequalities  exist.  Visible  light  re- 
flections from  the  retina  are  common  in  children  whose  eye  media,  as  a 
rule,  are  perfectly  transparent.  They  are  also  well  marked  in  dark 
evegrounds.  Retinal  reflexes  are  best  seen  with  a  concave  mirror  and 
feeble  IHumlnation.  Usually,  they  disappear  in  adult  life.  Wide  varia- 
tions exist  in  their  form  and  Intensity.  The  most  common  examples  con- 
sist; of  fine  silverv  lines  around  the  disc  or  flashes  of  pale  light  which 
plav  over  and  along  the  vessels.  Thev  are  a  normal  phenomenon  and  their 
presence  usuallv  indicates  a  healthy  fundus.  As  the  ophthalmoscopic 
mirror  is  moved,  retinal  reflexes  alter  their  shape  and  position  and  in 
this  v/ay  are  differentiated  from  patholoQ-Ic  changes  which  are  fixed.  The 
macula  being  a  concave  reflecting  surface  is  rich  In  reflexes  and  correspond- 
ingly difficult  to  examine,  because.  In  a  full  front  view,  both  macular 
and  corneal  reflexes  fall  dlrectlv  into  the  observer's  line  of  vision.  Ex- 
amination of  the  macula  is  accomplished  by  the  direct  method.  The 
subject  Is  told  to  look  at  the  light  In  the  mirror.  At  first  the  observer 
sees  little  or  nothing,  but  by  bringing  a  concave  lens  of  0.5 D.  into  the 
aperture  of  the  ophthalmoscope,  and  gazing  steadily  for  a  few  moments, 
th°  fo\'ea  will  gradually  appear.  The  brightest  macular  reflex  Is  that 
wh'ch  circles  around  the  walls  of  the  foveal  depression.  Viewed  from 
either  side,  this  reflex  appears  as  a  small  crescent  the  concavity  of  which 
is  always  towards  the  observer;  but  viewed  directly  from  in  front,  It 
appears  as  a  small  ring  with  a  diameter  equal  to  that  of  the  largest  vessel  on 


THE    NORMAL    FUNDUS.  15 

the  disc.  The  macula  is  darker  than  other  parts  of  the  fundus  and  the 
fovea  is  the  darkest  part  of  the  macula.  If  the  fundus  is  deeply  pigmented, 
the  macula  is  uniformly  dark,  but  if  the  retinal  epithelium  contains  little 
pigment,  the  macula  as  well  as  other  parts  of  the  fundus  may  be  stippled. 
It  is  important  to  differentiate  the  normal  stippling  of  a  light  macula 
from  the  patchy  discoloration  of  central,  syphilitic  choroiditis. 

Sterf.ogram  4.  The  Retinal  Vessels.  Left  eyeground  of  a  youth 
in  whom  the  retinal  vessels  are  normal  in  appearance,  and  their  method 
of  distribution  conforms  to  the  usual  scheme.  This  scheme  is  followed, 
in  a  general  way,  by  the  majority  of  eyes  but  varies  widely  in  individual 
cases.  The  disc  is  normal  and  contains  a  large  physiologic  excavation. 
Retinal  arteries  are  distinguished  from  veins  as  follows.  The  arteries 
are  narrower  than  the  veins  and  pursue  a  straighter  course.  The  surface 
of  the  retinal  vessels  reflects  a  central  streak  of  light  the  same  as  any 
polished  cylinder.  On  the  arteries  the  central  light  streak  is  broad,  bright 
and  reddish  in  color.  On  the  veins  it  is  narrow,  dull  and  white.  There 
has  been  much  inconclusi\e  discussion  as  to  whether  the  central  light 
streak  is  due  to  reflection  of  light  from  the  walls  of  the  vessel,  from 
the  column  of  blood  or  from  the  choroid.  The  distinctions  described  apply 
only  to  large  and  medium  sized  vessels.  The  character  of  a  small 
vessel  must  be  determined  by  tracing  it  back  to  its  source.  Retinal  vessels 
branch  dichotomouslv  and  diminish  in  size  from  the  disc  to  the  periphery. 
In  this  eye,  as  the  central  artery  emerges  from  the  disc  it  divides  into  the 
ascending  and  descending  papillary  arteries.  Frequently,  this  primary 
division  occurs  within  the  nerve.  The  ascending  artery  bifurcates  to 
form  the  superior  temporal  and  superior  nasal  arteries.  In  a  similar 
manner  the  descending  artery  splits  into  an  inferior  temporal  and  inferior 
nasal  branch.  The  veins  correspond  in  name  and  plan  of  distribution  to 
the  arteries.  In  the  majority  of  cases  the  artery  emerges  from  the  disc 
on  the  nasal  side  of  the  vein.  Several  small  vessels  emersfe  from  the 
edge  of  the  disc.  The  temporal  arteries,  which  are  slightly  larger  than 
the  nasal,  sweep  in  wide  curves  around  the  macula  to  which  they  send 
their  principal  branches.  Although  devoid  of  visible  vessels,  the  macular 
region  is  richly  supplied  with  capillaries  except  at  the  fovea  centralis 
which  is  absolutely  nonvascular.  In  fact,  retinal  vessels  in  this  location 
would  be  superfluous  inasmuch  as  the  fovea  consists  only  of  cones  and 
these  are  nourished  exclusively  by  the  choroid.  Springing  from  the 
temporal  side  of  the  disc  is  a  small  artery  distributed  to  the  region  between 
the  nerve  and  macula.     This  is  called  a  cilioretinal  artery  because  It  is 


1^  THE    FUNDUS    OCULI. 

supposed  to  arise  from  the  circle  of  Zinn  or  from  other  vessels  connected 
with  the  choroid.  A  true  cilioretinal  artery  is  recognized  by  the  manner  in 
which  it  hooks  over  the  edge  of  the  disc,  the  stem  being  directed  outward 
toward  the  choroid.  Exceptionally  these  arteries  appear  on  the  nasal 
side  of  the  nerve  (Strgm.  12).  The  existence  of  cilioretinal  veins  is 
doubtful.  If  they  exist  they  must  arise  directly  from  the  choroid  as  the 
posterior  ciliary  system  is  strictly  arterial.  The  majority  of  vessels  which 
emerge  from  the  margin  of  the  disc  arise  from  the  central  vessels  far  back 
in  the  nerve.  In  case  the  central  artery  is  occluded,  a  cilioretinal  artery  may 
permanently  preserve  a  small,  central  field  of  vision.  A  rare  vascular 
anomaly,  not  present  in  this  eye,  is  an  optico-ciliary  vessel  which  arises 
from  a  central  retinal  vessel  and  passes  to  the  edge  of  the  disc  into 
which  it  disappears  to  join  the  choroid.  The  retinal  vessels  are  dis- 
tributed onlv  to  the  inner  layers  of  the  retina.  The  external  layer  of 
the  retina  which  consists  of  the  rods  and  cones  is  nourished  by  the 
choroid.  The  trunk  vessels  are  the  central  artery  and  central  vein  of  the 
retina  Avhich  enter  the  eye  via  the  optic  nerve.  The  central  artery 
arises  from  the  ophthalmic  and  enters  the  optic  nerve,  in  company  with 
the  vein,  about  10-12  mm.  behind  the  eyeball.  The  central  vein  of  the 
retina  usually  empties  into  the  superior  ophthalmic.  The  ophthalmic  vein 
discharges  into  the  cavernous  sinus  but  also  communicates  with  the  facial 
vein.  This  communication  between  the  ophthalmic  and  facial  veins  is  of 
preat  importance  inasmuch  as  it  pro^'ides  a  free  outlet  for  the  retinal 
blood  should  the  cav^ernous  sinus  become  clogged.  In  the  head  of  the 
optic  nerve  the  central  retinal  arterv  anastomoses  with  vessels  from  the 
choroid  as  follows.  Just  before  the  short  ciliary  arteries  enter  the  choroid 
they  give  off  small  branches  which  unite  and  form  a  ring  in  the  sclera 
around  the  optic  nerve.  This  arterial  ring  is  known  as  the  circle  of  Zinn. 
Brnnches  from  Zinn's  circle  enter  the  head  of  the  nerve  and  anastomose 
with  twigs  from  the  central  artery  of  the  retina.  These  anastomoses  are 
little  more  than  capillaries;  nevertheless,  they  provide  a  feeble  collateral 
circulation  for  the  retina  in  closure  of  the  central  artery.  Vessels  from 
the  central  artery  supply  the  nerve  and  furnish  a  rich  capillary  plexus 
which  gives  color  to  the  optic  disc.  After  emerging  from  the  disc,  the 
retinal  vessels  form  a  strictly  terminal  system,  i.  e.,  they  do  not  anastomos-? 
with  other  vessels  or  with  each  other.  The  retinal  vessels  are  provided 
with  perivascular  sheaths  which  are  the  lymph  channels  of  the  retina. 

Pulse  Phenomena.     Pulsation  of  a  vein  upon  the  optic  disc  is  a 
physiologic  phenomenon  and  is  present  in,  approximately,  75  per  cent  of 


THE    NORMAL    FLxNDUS.  17 

normal  eyes.  Donders  explained  its  occurrence  as  follows.  The  cardiac 
systole  drives  the  blood  wa\'e  into  the  eye,  producing  momentary  increase 
in  intraocular  pressure,  and  this  rythmic  increase  of  pressure  may  be  suf- 
ficient to  empty  the  veins  on  the  disc.  Possibly,  venous  pulsation  may  be 
assisted  by  pulsatory  pressure  \'ariations  in  the  cavernous  sinus.  In  ac- 
cordance with  the  above  theories,  the  physiologic  venous  pulse  is  diastolic, 
/'.  e.,  the  veins  till  during  cardiac  diastole  and  empty  as  the  pulse  wave 
reaches  the  eye.  Another  explanation  of  the  venous  pulse  is  that  either 
in  the  ner\'e  or  on  the  disc,  the  artery  presses  against  the  vein  and  arterial 
pulsation  is  transmitted  by  contact.  This  would  explain  why  pulsation  is 
present  in  some  cases  and  absent  in  others,  and  why  it  frequently  is  limited 
to  a  single  vein.  Certainly,  pulsation  always  occurs  in  a  vein  which  is 
x'isibly  in  contact  with  an  artery.  \'enous  pulsation  can  be  produced  in 
normal  eyes  by  lightly  compressing  the  eye  and,  if  the  pressure  is  increased, 
pulsation  appears  in  the  artery  and  the  \ein  collapses.  Pulsation  of  the 
retinal  \'eins  may  be  of  pathologic  import.  A  positive  or  systolic  \enous 
pulse  may  occur  in  tricuspid  regurgitation.  In  aortic  regurgitation  both 
veins  and  arteries  pulsate.  Pulsation  in  the  retinal  arteries  always  is 
pathologic. 

The  systemic  blood  pressure  is  just  enough  higher  than  intraocular 
pressure  to  permit  a  smooth  flow  of  blood  through  the  retina  without 
visible  pulsation  of  the  arteries.  If,  however,  either  intraocular  pressure 
rises  above  or  systemic  blood  pressure  falls  below  its  relative  stand- 
ards, the  inflow  of  blood  is  hindered  and  the  artery  will  pulsate.  Two 
forms  o'f  arterial  pulsation  are  recognized,  (1)  the  pressure  pulse  which 
appears  only  in  the  arteries  on  the  disc.  It  occurs  in  the  heightened  eye 
pressure  of  glaucoma  and  in  the  lowered  blood  pressure  of  syncope, 
ischemia,  etc.  (2)  The  locomotion  pulse,  which  is  manifested  throughout 
the  retina  by  a  distinct  motion  of  the  arteries  seen  wherever  they  make  a 
quick  bend.  Locomotion  pulse  occurs  in  aortic  regurgitation,  where  it  is 
accompanied  by  a  pulsation  of  the  veins  and  by  an  alternate  flushing  and 
paling  of  the  disc.  It  may  occur  also  in  aneurism,  Basedow's  disease  and 
in  the  earliest  stage  of  diffuse  arteriosclerosis  when  the  arteries  are  relaxed 
(Sterg.  29).  Pulsatory  phenomena  may  be  simulated  by  slight  motions  on 
the  part  of  observer  or  subject,  and  the  diagnosis  of  abnormal  pulsation 
should  never  be  made  unless  It  is  distinct  and  unmistakable. 

Stereogram  5.  The  Optic  Nerve.  Optic  Disc  With  Physio- 
logic Excavation.  Right  eyeground  of  a  boy  12  years  of  age.  The 
case  was  selected  for  illustration  as  a  good  example  of  the  normal  disc. 

2 


18  IHE    FUNDUS    OCULI. 

The  outline  of  the  optic  disc  may  be  either  circular  or  oval.  The 
long  diameter  usually  is  vertical  or  slightly  tilted;  exceptionally,  it  lies 
transverselv.  1  he  general  color  of  the  disc  is  pink,  the  temporal  side 
being  lighter  in  color  than  the  nasal:  the  outline  is  somewhat  softened  by 
the  overlying  nerve  fibers,  but  is  well  defmed  in  normal  eyes.  The  majority 
of  nerve  libers  pass  to  the  nasal  side  of  the  retina;  hence,  this  side  of 
the  ner^'e  is  less  distinct  in  outline  than  the  temporal;  for  the  same  reason 
the  disc  is  darker  and  redder  on  the  nasal  side.  The  choroid  and  sclera 
usually  contain  an  excess  of  pigment  massed  around  the  edge  of  the  disc, 
forming  a  more  or  less  comulete  black  circle  calleci  the  choroidal  ring. 
Immediatelv  within  the  choroidal  ring  is  a  second  ring  of  white  connective 
tissue  which  Is  said  to  represent  the  distal  end  of  the  pial  nerve  sheath. 
It  is  generallv  called  the  scleral  ring  from  its  supposed  connection  with 
the  sclera.  In  the  stereogram  the  connective  tissue  ring  appears  well 
developed,  but  often  it  is  incomplete  or  absent.  The  lightest  colored 
part  of  the  nerve-head  is  centrally  located  and  marks  the  site  of  a  deep 
depression,  the  vascular  funnel,  a  canal  which  transmits  the  retinal  vessels. 
Usually,  the  vessels  appear  to  sink  and  fade  away  into  the  semitransparent 
tissue  which  nils  the  depression.  Not  infrequently,  however,  this  vascular 
funnel  is  \'ery  wide  and,  as  in  this  nerve,  occupies  a  large  part  of  the  disc 
forming  a  cavitv  know^i  as  a  physiologic  exca-vdlioji  (Figs.  21-22-23). 
The  formation  of  a  physiologic  e>?cavation  depends  upon  a  sclero- 
chcroidal  canal  which  is  too  large  for  the  nerve;  consequently,  the  nerve 
fibers  are  distributed  around  the  wall  of  the  canal  leaving  a  central 
space.  Phvsiologic  excavations  varv  in  shape  and  size.  The  most  fre- 
quent form  is  a  circular,  central  depression  (Stergm.  40).  An  oblique, 
funnel-shaped  excavation,  opening  towards  the  macular  side,  as  shown  in  the 
stereogram,  also  is  common.  Deep  excavations  extend  to  the  lamina  crib- 
rosa  the  perforations  of  which  appear  as  grey  dots  on  a  white  surface. 
The  main  retinal  vessels  usually  climb  up  the  nasal  side  of  a  physiologic 
excavat'.on.  The  macular  vessels,  however,  may  ascend  along  the  temporal 
side.  When  the  center  of  a  disc  occupies  a  lower  level  than  the  margin 
the  fact  mav  be  demonstrated  bv  the  test  of  parallactic  displacement  as 
folloAvs :  with  the  direct  method  of  examination,  if  the  observer  mo^•es 
his  head  from  side  to  side,  the  bottom  of  the  cup  will  follow  while 
the  edge  of  the  cup  will  move  in  the  opposite  direction. 

Anatomy.  The  optic  nerve  is  composed  chiefly  of  axis  cylinders 
or  neurons  from  the  ganglion  cells  of  the  retina.  These  neurons,  which 
constitute  the   inner  or  nerve  fiber  layer  of  the    retina,    converge   to   the 


Fig.   22.  Fig.   2:1. 

Figs.  21,  22,  23 — Normal   Di.scs  with   Physiologic   Excavations. 


THE    NORMAL    FUNDIS.  19 

scleral  foramen  where  they  form  the  optic  nerve  and  pass  to  the  brain. 
The  function  of  the  optic  nerve  is  to  convey  to  the  higher  cerebral  centers 
the  impressions  gathered  by  the  retina.  The  nerve  also  contains  fib^^rj 
which  con\'ey  impulses  from  the  brain  to  the  eye.  The  optic  nerve  miy 
be  divided  into  four  parts:  (1)  I  he  iutracrcui'uil ,  which  is  about  1  cm. 
in  length  and  extends  from  the  chiasm  to  the  inner  opening  of  the  optic 
canal.  This  portion  of  the  nerve  is  subject  to  pressure  froin  tumors  a  id 
to  constriction  from  organized  inflammatory  exudates;  (2)  fhe  intra- 
cauaUuiihir  di\i3ion,  which  lies  within  the  optic  canal.  This  canal  co  i- 
sists  of  a  bony  portion  4  mm.  in  length  and  a  fibrous  portion  which  extends 
into  the  cranial  qavnty  for  a  distance  of  2  mm.  Only  the  optic  ner\e  and 
ophthalmic  artery  pass  through  the  optic  canal,  and  it  is  said  that  sclerosis 
oi  tlie  artery  may  cause  pressure  atrophy  of  the  nerve.  The  nerve  is 
injured  in  the  canal  also  by  periostitis,  thickening  of  the  bone  and, 
especially,  by  fracture  of  the  orbit  which  tends  to  pass  through  the 
f^'^tic  foramen.  (3)  The  orhiUil  divisioii,  about  30  mm.  in  length,  includes 
that  portion  of  the  nerve  which  extends  from  the  optic  foramen  to  the 
eye'all.  This  section  ol  the  nerve  is  very  lax  and  pursues  an  S-shaped 
course  which  permits  free  rotation  of  the  eve  without  rendering  the  nerve 
taut.  (4)  The  intraociihir  di\'ision  of  the  nerve  comprises  that  portion 
of  the  nerve  which  pierces  the  sclera  and  choroid.  It  measures  about 
1.25  mm.  in  length.  The  so-called  scleral  foramen,  through  which  the 
nerve  passes  into  the  eye,  consists  of  numerous  small  holes  in  the  inner 
layers  of  the  sclera,  forming  a  sieve-like  partition  known  as  the  lamina 
crihro.ui.  The  externa'  fibers  of  the  sclera  do  not  enter  into  the  formation 
of  the  lamina  but  are  reflected  backward  and  become  part  of  the  dural 
sheath  of  the  nerve.  Inasmuch  as  the  lamina  cribrosa  is  composed  of 
unyielding  fibrous  tissue,  it  is  instrumental  in  causing  congestion  and 
edema  of  the  nerve-head.  That  portion  of  the  optic  nerve  situated  in  front 
of  the  lamina  cribrosa  is  ophthalmoscopically  visible  and  constitutes  the 
optic  disc  or  papilla.  The  name  "  papilla  "  is  a  survival  of  early  ophthal- 
moscopic days,  when  the  head  of  the  nerve  was  erroneously  supposed  to 
project  into  the  eye.  In  normal  eyes  the  optic  disc  is  level  with — or  below 
the  surrounding  fundus.  In  the  optic  canal  and  orbit  the  nerve  is  enclosed 
by  extensions  of  the  cerebral  membranes,  i.  e.,  the  dura,  arachnoid  and  pia. 
The  central  artery  and  vein  of  the  retina  enter  the  nerve  about  10  mm. 
behind  the  globe:  therefore,  compression  or  injury  of  the  nerve  posterior 
to  this  point  does  not  affect  retinal  circulation. 


Chapter  III. 
CONGENITAL  ANOMALIES. 

Stereogran[  6.  CoLOBOiMA  OF  THE  CiiOROU).  Right  eyeground  of 
boy  12  vears  of  age.  Left  eye  is  normal.  On  his  right  side  the  patient 
has  rudimentary  auricle,  paresis  of  the  seventh  nerv^e,  coloboma  of  the  iris, 
lens  and  choroid.  Contrary  to  the  rule,  the  coloboma  of  the  choroid  is 
nearly  circular  in  outline.  It  presents  an  extensiv^e,  bluish-white  surface 
extending  from  the  center  ol  the  disc  downward  and  outward.  The  out- 
ward de^iation  appears  to  be  caused  by  rotation  of  the  eyeball.  The 
lovver  halt  of  the  optic  disc  is  included  in  the  imperfection.  The  adjacent 
fundus  is  strongly  pigmented.  The  coloboma  forms  a  craterform  de- 
pression in  the  fundus  about  2  mm.  (6.D.)  below  the  normal  level.  On 
the  floor  of  the  depression  are  two  small,  oval  ectasias.  The  neighboring 
fundus,  including  the  optic  nerve,  appears  to  slope  into  the  pit.  The  retinal 
vessels  make  wide  detours  and  skirt  along  the  margin  of  the  coloboma  as 
though  they  had  been  pushed  aside.  The  defect  is  covered  by  retinal 
tissue,  as  shown  by  a  medium-sized  retinal  vessel  which  crosses  from 
above  downward  and  outward.  The  branching  vessel  v>^hich  enters  the 
coloboma  through  the  floor  above,  probably  is  a  ciliary  artery,  while  the 
vascular  loops  which  push  in  along  the  edge  are  from  the  neighboring 
choroid.  There  appears  to  be  dim  perception  of  light  throughout  the 
cclobomatous  area,  indicating  the  presence  of  retinal  elements. 

Coloboma  of  the  choroid  is  a  congenital  gap  in  the  membrane  due  to 
arrested  development.  The  sclera  is  exposed  in  the  defect  and  presents  a 
brilliant  white  or  bluish-white  surface  on  which  spots  of  pigment  are  not 
uncommon.  The  typical  coloboma  of  the  choroid  is  situated  near  the 
median  line  below  the  optic  nerve.  The  smallest  coloboma  I  ever  saw  was 
about  twice  the  diameter  of  the  optic  disc  (colored  plate  1),  and  the 
largest  covered  nearly  half  the  fundus.  As  a  rule,  a  bridge  of  choroidal 
tissue  separates  the  defect  from  the  disc,  but  not  infrequently  the  nerve  also 
is  involved.  Coloboma  of  the  choroid  usually  occurs  in  connection  with 
coloboma  of  the  iris  or  other  malformation  (F\g  24).  Coloboma  of  the 
lens  is  often  associated  but  usually  is  overlooked,  as  it  may  consist  of  only 
a  slight  flattening  at  the  lower  edge.     Frequentlv,  the  cornea  is  vertically 

20 


Fig.  24. 
CololKima  of  the  Upper  Lid  vvliich   was  associated  with   Coloboma  of  the   Choroid. 


CONGENITAL    ANOMALIKS. 


21 


oval  or  pointed  below.  Researches  have  shown  that  coloboma  of  the 
choroid  is  eminently  hereditary.  The  typical  outline  of  the  imperfection 
is  a  vertical  oval  or  an  obtusely  angled  triangle  with  the  apex  toward  the 
disc.  Rarely  it  is  round  or  the  long  diameter  is  directed  transversely.  In 
some  fetal  eyes  both  choroid  and  sclera  are  so  defective  that  the  intraocular 
tissues  grow  outward  and  form  a  large  sac  below  the  eye,  while  the 
eyeball  remains  very  small  (microphthalmus  with  sac).  Eyes  with  colo- 
boma of  the  choroid  usually  are  small.  The  refraction,  however,  is 
myopic  owing  to  the  ectasia  of  the  sclera.  As  a  rule,  some  perception  of 
light  exists  in  the  colobomatous  region. 

The  diagnosis  of  typical  coloboma  of  the  choroid  presents  no  diffi- 
culties, but  atvpical  cases  cannot  always  be  differentiated  from  old  cicatrices. 


Coloboma  of  tiik  Choroid. 

Congenital    malformation. 

Is  below  the  level  of  the  fundus. 

Usually,  a  single  defect. 

Situated  below  nerve  near  median 
line. 

Always  much  larger  than  the  optic 
disc. 

Outline  symmetrical.  Usually,  ver- 
tically oval. 

Frequently  associated  with  colo- 
boma of  iris  nerve  or  other  ocu- 
lar malformation. 

Usually  represented  by  relativ:." 
scotoma  in  visual  field. 

Seldom  affects  both  eyes. 


CiCATRLX  OF  THE   CHOROID. 

Results  from  disease  or  trauma. 

Is  level  with  fundus. 

Usually,  more  than  one  lesion. 

Situated   anywhere   in   the    fundus. 

May  be  of  any  size. 

Usually    circular    or    irregular    in 
outline. 

Not  associated  with  congenital  de- 
fects. 

Represented  by  absolute  scotoma. 
Frequently  present  in  both  eyes. 


In  typical  rupture  of  the  choroid  a  crescentic  area  of  sclera  is  exposed, 
concentric  with  the  optic  disc.  Rupture  is  further  differentiated  from  colo- 
boma by  a  history  of  injury. 

Stereogram    7.      Coloboma    of    Choroid    and    Optic    Nerve.* 

*    Russian  girl  1 1  years  of  age.     Right  eye  normal.     Left  eye  presents  an 

enormous  coloboma  of  the  choroid  which  includes  the  lower  third  of  the 


From  the  service  of  Dr.  J.  Scott  Wood,  Brooklyn  Eye  and  Ear  Hospital. 


22  THE    FUNDUS    OCULI. 

fundus,  the  optic  nerve  and  the  macuhi.  Its  posterior  extremity  extends 
beyond  the  optic  nerve  and  its  anterior  border  beyond  the  limits  of  the 
ophthalmoscopic  held.  The  edge  of  the  coloboma  is  sharp  cut  as  though 
the  choroid  had  been  punched  out  with  a  die.  It  is  framed  by  a  border 
of  dense  pigment,  and  pigment  anomalies  are  seen  in  the  surrounding 
fundus.  The  floor  of  the  coloboma  is  about  1  mm.  (3.D.)  below  the 
level  of  the  fundus.  Its  surface  is  broken  by  several  pits  and  channels. 
The  entire  coloboma  is  covered  with  loose,  web-like  tissue,  evidently 
imperfectly  formed  retina.  Posteriorly,  this  tissue  is  thick  and  contains 
retinal  vessels;  anteriorly,  it  is  tenuous  and  transparent.  Filmy  mem- 
branes and  dense  tangles  of  tissue  containing  pigment  extend  out  from  the 
margin,  and  web-like  bands  stretch  across  the  gap.  The  optic  disc  is 
not  apparent,  but  its  site  is  indicated  by  the  retinal  vessels  which  con- 
■verge  to  a  point  in  the  dense  tissue  of  the  coloboma.  These  vessels  can 
be  traced  from  their  origin,  across  the  pigmented  border,  out  into  the 
retina.  In  the  lower  part  of  the  coloboma,  the  lar;^2  vessel  which  emerges 
from  the  pigmented  bord.r  and  extends  across  the  pit  is  known,  from  its 
manner  of  branching,  to  be  from  the  choroid. 

Pathogenesis.  Various  theories  have  been  advanced  to  explain  colo- 
boma of  the  choroid,  none  of  which  have  been  established.  The  situation 
of  the  defect,  along  the  median  line  belcv/  the  disc,  corresponds  to  the 
fetal  cleft  in  the  seconciary  optic  vesicle.  Hence,  it  was  assumed  that 
coloboma  of  the  choroid  resulted  whenever  this  cleft  failed  to  unite. 
Unfortunately  for  this  simple  theory,  the  secondary  optic  vesicle  represents 
the  retina  and  the  choroid  does  not  develop  from  this  layer  ( Fig.  25  ) .  Fur- 
thermore, the  secondary  optic  vesicle  is  situated  internal  to  the  choroid 
and  has  been  found  perfectly  united  in  many  cases  of  coloboma  of  the 
choroid.  The  choroid  develops  from  the  blastoderm,  forms  a  vascular 
coat  external  to  the  retina,  and  possesses  no  fetal  cleft  (Fig.  26). 
Another  argument  against  the  theory  that  coloboma  is  due  to  nonclosure 
of  the  fetal  cleft  is  the  occasional  occurrence  of  coloboma  on  one  side 
of,  or  even  abov^e,  the  nerve,  v.  Hippel  calls  attention  to  the  influence  of 
the  vitreous  upon  the  formation  of  coloboma.  If  the  vitreous  is  scanty 
the  retina  does  not  get  spread  out  but  lies  in  folds  some  of  which  may 
fall  into  the  fetal  cleft.  This  accident  or  the  persistence  of  mesoblastic 
tissue  in  the  cleft,  is  sufl'icient  to  prevent  development  of  the  choroid 
at  this  point.  Treacher  Collins  and  W.  Lang  attribute  all  cases  of 
choroidal  coloboma  to  abnormal  adhesions  between  the  mesoblastic  cho- 
roid  and   epiblastic   retina.      Prenatal    inflammation   has   been   widely   ac- 


Fig.  23. 


Secondary  Optic  Vesicle.     A,  exterior  layer;  P,  interior  layer;  M,  mesoderm;  L.  lens. 


Fig.  26. 
Development  of  the   Choroid  from  the  Blastoderm. 


CONGENITAL    ANOMALIES.  23 

cepted  as  a  cause  for  coloboma,  but  microscopic  examinations  have 
failed  to  discov^er  any  evidence  of  inHammatory  action.  Furthermore, 
typical  coloboma  is  an  hereditary  anomaly  and  nearly  always  occurs 
in  a  certain  situation,  two  facts  which  are  inconsistent  with  either  accidental 
or  inflammatory  origin.  For  certain  atypical  cases,  however,  an  ex- 
planation may  be  found  in  prenatal  inflammation.  Landman  reported  a 
case  of  absence  of  the  choroid  in  both  eyes  and  attributed  the  imperfection 
to  embryologic  obliteration  of  the  posterior  ciliary  arteries.  Atypical 
coloboma,  /'.  e.,  occurring  elsewhere  than  below  the  nerve,  has  been  ex- 
plained on  the  supposition  that  certain  eyes  may  possess  two  fetal  clefts, 
a  condition  which  has  been  observed  in  some  of  the  lower  animals. 
Notwithstanding  our  present  inability  to  explain  the  method  in  which 
coloSoma  of  the  choroid  is  formed,  its  location  alon'j;  the  line  of  the  fetal 
cleft  is  a  potent  argument  that  some  tault  in  the  cleft  is  instrumental  in 
arresting  development  of  the  choroid  at  this  place.  Microscopic  examina- 
tions of  colobomata  of  the  choroid  show  that  the  defect  is  almost  inxariably 
covered  by  more  or  less  imperfectly  formed  retina  which  may  consist  of 
all  layers  or  of  only  a  single  layer,  the  membrana  limitans  interna  (Figs. 
27-28).  Absence  of  the  retina  o\er  a  coloboma  cannot  be  determined  by 
ophthalmoscopic  examination. 

Stekf.ogram  8.  Coloboma  of  the  NLacula.  Right  eyeground  of  a 
girl  16  years  of  age.  Left  eye  normal  in  all  respects.  Right  eye  possesses 
only  peripheral  ^'ision.  Central  light  perception  cannot  be  demonstrated. 
The  macular  region  is  occupied  by  a  horizontally  oval  defect,  of  about 
two  discs'  diameter,  evidently  a  coloboma  of  the  choroid.  The  fundus 
contains  no  other  imperfection  or  evidence  of  disease.  The  floor  of  the 
coloboma  consists  of  bluish-white  sclera,  depressed  about  0.3  mm.  (l.D.) 
below  the  plane  of  the  fundus.  Dense  masses  of  pigment  encircle  the 
defect  and  cross  its  surface. 

The  great  majority  of  choroidal  colobomata  follow  the  line  of  the 
fetal  cleft  as  shown  in  stereograms  6-7.  Occasionally,  however,  atypical 
cases  of  this  malformation  occur  situated  elsewhere  than  below  the  disc. 
Among  others,  the  following  examples  of  atypical  coloboma  have  been 
reported:  coloboma  of  choroid  and  iris  outward;  coloboma  of  choroid 
and  iris  inward;  of  choroid  in  one  direction  and  of  iris  in  another;  bilateral 
coloboma  outward;  coloboma  upward,  etc.  The  most  common  atypical 
coloboma  of  the  choroid  occurs  at  the  macula.  In  this  situation  the  defect 
usually  is  elliptical,  the  long  diameter  directed  horizontally;  rarely  it  is 
round,    rhomboid   or   triangular   In   outline.      Its   size    ranges    from    two 


24  THE    FUNDUS    OCULI. 

to  six  disc  diameters.  Genuine  cases  present  the  appearance  of  typical 
colohonia,  ;'.  e.,  the  margin  is  sharply  marked  and  pigmented,  the  surface 
usually  is  depressed  and  may  be  crossed  by  vessels  or  pigment. 

f)i.\(;xosis.  Natal  hemorrhages  or  ruptures  of  the  choroid  may  re- 
sult in  cicatrices  that  cannot,  with  certainty,  be  differentiated  from  con- 
genital coloboma.  An  associated  coloboma  of  the  iris  is  the  best  evidence 
that  the  choroidal  defect  is  a  malformation  and  not  a  cicatrix.  Cases  in 
which  the  fundus  contains  other  lesions,  especially  those  of  chorioretinitis, 
cannot  be  accepted  as  genuine.  There  is  sometimes  a  tendency  for  colo- 
bomatous  eyes  to  rotate  on  their  anteroposterior  axis  (torsion)  ;  therefore, 
the  position  of  the  eye  should  be  determined  before  deciding  that  the 
direction  of  a  coloboma  is  atypical. 

Stkreogram  9.  Coloboma  of  Optic  Nerve  and  Nerve  Sheath.* 
Right  eyeground  of  a  woman  27  years  of  age.  Vision  is  20/50.  The  oph- 
thalmoscope rev^eals  a  colobomatous  formation  involving  the  optic  nerve. 
The  area  of  the  disc  appears  greatly  enlarged  and  depressed.  The  de- 
pression is  surrounded  by  a  white  zone  of  exposed  sclera,  level  with  the 
surrounding  fundus  from  which  it  is  separated  by  a  ring  of  black  pigment. 
The  pit  ot  the  coloboma  is  shallow  above  and  gradually  slopes  downward 
and  backward,  terminating  below  in  a  pouch-like  cavity  resembling  a  glau- 
comatous excavation  into  which  the  vessels  dip  and  disappear.  Details 
are  indistinct  owing  to  a  semitransparent  tissue  which  fills  the  depression. 
The  surface  of  the  coloboma  may  be  divided  into  three  parts  as  follows: 

(1)  the  nerve  head,  represented  by  an  oval,  pinkish  disc,  situated  above; 

(2)  the  surrounding  floor  and  walls  of  the  pit  which  possess  a  bluish-white 
color  and  represent  a  defect  in  the  scleral  canal  and  sheaths  of  the  nerve 

(coloboma  of  the  nerve  sheaths)  ;  (3)  the  white  zone  and  pigment  ring. 
The  line  of  pigment  marks  the  l!n":it  of  the  choroid;  therefore,  the  white 
zone  is  a  defect  or  coloboma  in  the  choroid.  The  greatest  depth  of 
the  coloboma  is  1.3  mm.   (4.D.). 

Several  malformations  involving  the  head  of  the  nerve  are  grouped 
under  the  name  of  "  coloboma  of  the  nerve."  These  are  :  {a)  Coloboma  of 
the  choroid  Avhich  includes  the  nerve;  {h)  Coloboma  of  the  nerve  sheaths 
formed  by  enlargement  of  the  scleral  canal  and  dilatation  of  the  sheaths. 
Tn  these  cases  the  sheaths  are  not  closed  In  front  by  normal  sclera,  neither 
is  the  coloboma  co\ered  by  choroid;  (r)  Coloboma  limited  to  the  nerve. 
Tn  fetal  life  the  lower  surface  of  the  oDtic  nerve  contains  a  cleft  through 
which  the  central  vessels  enter.     Failure  of  this  cleft  to  close  constitutes 

*  F-Mm  the  service  of  Dr.  David  Webster,  Manhattan  Eye,  Ear  and  Throat 
Hospital. 


Fig-  27.  Fig.  28. 

Fig.  27 — Coloboma  of  Choroid.     The   Retina  continued  over  the  defect. 
Fig.  28— Coloboma  of  Choroid.    The  Retina  dips  into  and  lines  the  defect. 


Fig.  29. 
Coloboma  of  Optic  Nerve  and  Sheaths. 


CONGENITAL    ANOMALIES.  25 

a  true  coloboma  of  the  nerve.  On  closure  of  the  fetal  cleft,  if  the  retinal 
vessels  are  excluded  from  the  nerve  they  are  replaced  by  ciliary  vessels 
which  enter  the  eye  around  the  pseudo-disc,  usually  below.  Fuchs  regards 
the  common  inferior  crescent  of  the  optic  disc  (Stergm.  10)  as  a  feebly 
developed  coloboma,  a  view  which  has  not  been  universally  accepted.  The 
scheme  of  distribution  of  the  retinal  vessels  in  coloboma  of  the  nerve  has 
been  divided  by  Casper*  into  three  classes  as  follows: 

( 1 )  All  vessels  emerge  from  the  lower  part  of  the  coloboma  or 
pseudo-disc. 

(2)  Vessels  emerge  from  near  the  center  of  the  disc  and  are  dis- 
tributed In  a  normal  manner. 

(3)  Vessels  enter  the  eye  around  the  periphery  of  the  coloboma 
(ciliary  vessels) . 

Colobomata  of  the  nerve  and  its  sheaths  present  the  most  varied 
ophthalmoscopic  pictures.  The  defective  area  varies  In  size  from  two 
to  twentv  disc  diameters.  Usually,  the  depressed  portion  is  deepest 
below.  I.ocal  £CtasijF  in  the  walls  of  the  coloboma  are  frequently  ob- 
served. True  colobomata  limited  to  the  nerve  occur  as  holes  In  the 
disc,  quite  different  from  a  glaucomatous  or  physiologic  excavation.  The 
"  punched-out  "  hole  sometimes  seen  in  the  disc  Is  regarded  by  Lindsay 
[ohnsont  as  a  vestigal  trait.  A  similar  formation  occurs  normally  In 
the  optic  ner\e  of  the  elephant.  Coloboma  of  the  nerve  usually  Is 
associated  with  coloboma  of  choroid,  Iris,  lens  or  some  other  ocular  mal- 
formation. It  is  said  to  exist  in  20  per  cent  of  microphthalmic  eyes. 
Vision  may  be  nearly  normal;  usually,  however.  It  is  very  defective.  En- 
largement of  the  normal  blind  spot  corresponds  to  the  size  of  the  coloboma. 

I  found  the  following  example  of  the  nerve  and  sheaths  in  the  eye  of 
an  Infant  who  died  three  weeks  after  birth  :|  Diameter  of  the  globe  was 
14  mm.  Microscopic  exam'mat'wn.  Anterior  hemisphere  contains  no  mal- 
formation. Immediately  below  the  head  of  the  optic  nerve  Is  a  large  pit 
formed  by  bulging  of  the  dural  sheith  a-id  external  fibers  of  the  sclera. 
Most  of  the  internal  layer  of  scleral  fibers  stop  abruptly  at  the  edge  of 
the  pit.  At  one  side,  however,  a  bundle  of  fibers  continues  for  a  short 
distance  over  the  pit  and  then  sharply  bends  forward  to  form  a  column  of 
connective  tissue  (Fig.  29)  in  the  vitreous.  Evndently,  these  strands  of 
connective  tissue  represent  the  lamina  cribrosa  which  has  made  an  abortive 

*  Casper.  Dissertation.  Bonn,  1887. 
t  Lindsay  Johnson,   Pocket   Atlas,  etc. 

%  From  the  lahoratory  of  the  New  York  Post  Graduate  IVIedical  School  and 
Hospital. 


26 


THE    FUNDUS    OCULI. 


attempt  to  bridge  the  chasm.  The  optic  nerve  contains  the  retinal  vessels 
and  is  well  formed  except  at  its  ocular  end  where  it  dips  downward  Into 
the  colobomatous  excavation  (Fig.  30).  The  retina  preserves  its  normal 
structure  until  It  arrives  at  the  pit  into  which  it  enters  and  forms  a 
tangled  meshwork  above  the  nerve  fibers.  The  choroid  ends  at  a  short 
distance  from  the  edge  of  the  pit,  leaving  a  zone  of  sclera  uncovered. 
Dia(;nosis.  Coloboma  limited  to  the  nerve  appears  as  holes  or  clefts 
in  the  disc,  usuallv  situated  below.  A  small  coloboma  limited  to  the  nerve 
and  sheaths  may  present  a  superficial  resemblance  to  glaucomatous  ex- 
cavation. Reference  to  the  following  table  will  show  the  essential  points 
in  which  the  two  conciitions  differ: 


Coloboma  of  Optic  Nerve  and 
Sheaths. 

In  the  absence  of  congenital  mal- 
formations, the  external  aspect 
of  the  eye  Is  normal. 

Eye  tension  normal. 

Congenital  malformation. 

Non-progressive. 

Visual  field  shows  only  enlargement 
of  normal  blind  spot. 

Usually  unilateral. 

No  pain  or  inflammation. 

Vessels  not  diseased  but  are  distrib- 
uted  anomalously. 

Anatomic  landmarks  disturbed. 

Disc  varies  in  color.  Lamina  crib- 
rosa  not  seen. 


Glaucoma. 


The  eye  presents  the  glaucomatous 
aspect. 

Tension  Increased. 

Disease  of  adult  life. 

Progressive. 

Characteristic  contraction  ^f  nasal 
field. 

Both  eyes  affected  though  in  vary- 
ing degrees. 

Even  cases  of  simple  glaucoma 
may,  at  times,  suffer  pain. 

Vessels  diseased  but  are  normally 
distributed. 

Landmarks  undisturbed. 

Disc  grey  in  color  and  atrophic. 
Lamina  markings  usually  dis- 
tinct. 


Stereogram  10.  Inferior  Optic  Crescent  (Congenital  Crescent; 
Inferior  Staphyloma:  Fuchs'  Coloboma:  Conus  nach^unten).  Right 
eyeground  of  a  girl  19  years  of  age.  Similar  conditions  exist  In 
'-nth  eyes.  ^  Has  hyperopic  astigmatism.  With  correcting  glasses  the 
vision  remains  slightly  subnormal.  The  optic  disc  presents  the  only  ab- 
,....-  pi. ;-,,  j,  ^.i^g  fu-^dus.  The  nerve  head  is  redden-d.  Adjoining  the 
disc  below.  Is  a  brond,  white  crescent.  The  crescent  is  not  depressed  and 
the  retinal  vessels  cross  its  surface  without  bending.     The  pigment  which 


CONGENITAL    ANOMALIES.  27 

surrounds  the  top  and  sides  of  the  disc  is  continued  around  the  crescent, 
so  that  the  latter  appears  to  form  part  of  the  nerve  head. 

The  above  description  is  applicable  to  all  well  developed  cases  of 
congenital,  inferior  conus  or  crescent  of  the  disc.  A  congenital  crescent 
may  appear  around  any  portion  of  the  nerve  but  the  great  majority  are 
directed  downward.  In  physiologic  excavation  the  cup  always  opens 
toward  a  conus  when  the  two  conditions  are  associated.  When  a  disc 
with  inferior  crescent  also  has  a  connective  tissue  ring,  the  ring  and  crescent 
are  continuous.  As  a  rule,  inferior  crescent  occurs  in  eyes  with  refractive 
errors  and  slightly  subnormal  vision. 

Although  inferior  crescent  is  a  common  condition,  its  anatomic  struc- 
ture is  undetermined.  Probably,  similar  ophthalmoscopic  pictures  are  pro- 
duced by  differing  conditions.  The  relation  of  inferior  to  temporal 
(myopic)  crescent  is  uncertain;  at  all  events,  they  are  clinically  regarded  as 
independent  conditions.  Temporal  crescent  is  almost  constant  in  myopia, 
but  inferior  crescent  is  common  in  hyperopia  and  hyperopic  astigmatism. 
Fuchs  regards  the  inferior  crescent  as  an  incomplete  coloboma.  If  so, 
it  differs  from  undoubted  colobomata  in  seldom  being  associated  with  other 
formative  defects.  Elschnig  would  limit  the  term  "  coloboma  "  to  those 
defects  which  are  depressed  below  the  level  of  the  disc.  He  regards  all 
crescents  as  congenital  even  though  they  progressively  increase  in  size. 
Frost  states  that  inferior  crescent  is  an  unperforated  portion  of  the  lamina 
cribrosa ;  in  support  of  which  he  points  out  that  conus  always  occurs 
within  the  limits  and  at  the  expense  of  the  disc.  The  reddened  disc  he 
attributes  to  crowding  of  the  nerve  fibers  into  insufficient  space.  Other 
theories  of  origin  are:  (1)  exposed  sclera;  (2)  exposed  inner  surface  of 
nerve  sheath;   (3)   congenital  absence  of  the  choroid. 

Stereogram  11.  Opaque  Nerve  Fibers  (Medullated  Nerve 
Fibers).  Left  fundus  oculi  of  man,  48  years  of  age.  Similar  ophthalmo- 
scopic pictures  are  presented  by  the  two  eves.  Central  vision  in  each  eye 
is  normal.  The  visual  fields  show  enlargement  of  the  physiologic  blind 
spot. 

Left  Eyeground.  Extending  into  the  retina  from  the  upper  and 
lower  margins  of  the  disc  are  large,  white,  striate  patches  terminating  In 
delicate  filaments,  resembling  brush-strokes  of  white  enamel.  These  patches 
are  typical  opaque  nerve  fibers.  Additional  fibers  are  scattered  along  the 
inner  and  outer  margins  of  the  nerve  and  a  solitary  wisp  is  observed 
around  the  inferior  temporal  artery.  The  fibers  become  opaque  first 
near  the  periphery  of  the  disc.     They  pass   behind  and  in   front  of  the 


28  THE    FUNDUS    OCULI. 

retinal  vessels,  obscuring  or  completely  hiding  them.  Although  the 
vessels  appear  embedded  in  solid  tissue,  their  circulation  is  in  no  way 
disturbed.  The  disc  appears  unduly  red,  but  this  is  an  artefact  produced  by 
contrast  with  the  brilliant  white  nerve  fibers. 

The  nerve  fibers  which  compose  the  optic  nerve  have  no  neurilemma, 
but  each  fiber  is  medullated,  i.  e.,  it  is  enclosed  by  the  fat-like  substance 
known  as  the  myelin  sheath.  At  the  lamina  cribrosa  the  nerve  fibers  lose 
this  myelin  sheath  and  only  naked  axis  cylinders  enter  the  eye.  The  utility 
of  this  change  is  obvious,  inasmuch  as  medullated  fibers  are  opaque, 
while  non-medullated  fibers,  designed  for  the  retina,  are  perfectly  trans- 
parent. Tt  may  happen,  as  in  this  case,  that  certain  nerve  fibers,  after 
passing  into  the  eye,  are  again  invested  with  a  myelin  sheath  or  the  sheath 
may  be  continued  from  the  nerve,  through  the  lamina  into  the  retina. 
In  the  retina  the  opaque  fibers  are  ophthalmoscopically  visible  as  brilliant, 
bluish-white  striae.  Opaque  nerve  fibers  are  almost  constant  in  the  rab- 
bit's retina,  and  in  man  they  occasionally  occur  as  a  physiologic  anomaly. 
Throughout  the  nervous  system  development  of  myelin  sheaths  proceeds 
from  center  to  periphery.  As  they  form  late  in  fetal  life,  medullation 
of  the  optic  nerve  is  not  completed  until  a  month  or  two  after  birth; 
consequentlv,  opaque  nerve  fibers  in  the  retina  are  not,  strictly  speaking, 
congenital.  Intraocular  medullated  nerve  fibers  present  a  striking  oph- 
thalmoscopic picture  frequently  mistaken  by  the  novice  for  a  pathologic 
condition.  In  the  great  majority  of  cases  opaque  fibers  are  attached  to 
the  optic  nerA^e,  usually  at  the  upper  or  lower  margin.  Very  rarely  they 
are  confined  within  the  borders  of  the  disc.  In  other  cases  the  disc  and 
neighboring  fundus  are  normal,  while  a  distant  patch  of  opaque  nerve 
fibers  appears  on  the  surface  of  the  otherwise  transparent  retina.  Such 
isolated  fibers  usuallv,  but  not  invariably,  appear  thin  and  translucent. 
The  number  of  opaque  nerve  fibers  varies  in  different  cases  from  an  in- 
significant patch,  which  may  escape  observation,  to  an  enorrrious  white 
zone  completely  encircling  the  disc.  When  few  in  number  and  equally 
distributed  around  the  nerve  they  resemble  the  retinal  striation  of  in- 
cipient neuritis. 

One  or  both  eyes  may  be  affected,  more  frequently  both.  Bilateral 
cases  seldom  are  symmetrically  developed.  Blindness  exists  in  those  areas 
occupied  by  opaque  nerve  fibers.  If  fibers  are  abundant  on  the  macular 
side  of  the  nerve  vision  may  be  reduced,  but  ordinarily  they  are  so 
situated  as  to  cause  no  functional  disturbance.  The  anomaly  appears  to 
be  hereditary  and  frequently  affects  several  members  of  a  family. 


CONGENITAL    ANOMALIES.  29 

Diagnosis.  Patches  of  opaque  nerve  fibers  disconnected  from  the 
disc  are  especially  apt  to  hs  mistaken  for  a  manifestation  of  disease.  They 
are  recognized  by  their  filamentous  structure,  radial  direction  and  by  the 
manner  in  which  they  run  over  and  under  vessels  without  affecting  the 
circulation.  As  distinguished  from  striations  around  a  choked  disc,  opaque 
nerve  fibers  are  sharply  circumscribed  and  brllliantlv  white:  Furthermore, 
they  are  not  accompanied  by  swelling  of  the  nerve-head,  engorgement  of 
veins,  hemorrhage  or  edema,  all  of  which  belong  to  the  clinical  picture 
of  choked  disc.  Opaque  nerve  fibers  are  readilv  differentiated  from  cho- 
roicial  disease  by  their  superficial  situation.  In  albuminuric  retinitis  small, 
fluffy,  striate  exudates  may  occur  between  the  nerve  fibers,  but,  usually, 
they  are  accompanied  by  other  exudates  and  bv  N'nscular  degeneration. 
The  large  "  snowbank  "  exudate  which,  in  albuminuric  retinitis,  sometimes 
involves  the  optic  nerve,  never  presents  th^  compact,  white,  shining  surface 
of  opaque  nerve  fibers.  Retinal  light  reflexes  differ  from  opaque  nerve 
fibers  in  that  they  arc  widely  distributed  and  they  appear  and  disappear 
on  changing  the  position  of  the  ophthalmoscopic  mirror. 

Strrkogkam  12.  Prkfapillarv  Rftinai,  Aktkrv.  Left  eyeground 
of  a  bov  which,  among  other  phvsiologic  variations,  presents  a  beautiful 
vascular  anomalv.  Retinal  and  choroidal  pigment  is  scantv  •'hioughout  the 
fundus.  The  region  around  the  disc  which  usually  contains  an  excess  of 
pigment,  is  almost  completely  depigmentLnl.  A  curious  "  pseudo-conus  " 
exists  on  both  sides  of  the  nerve.  The  eye  is  hyperopic  and,  as  is  common  in 
this  condition,  the  retinal  vessels  are  rather  tortuous.  Three  vessels  which 
emerge  from  the  margin  of  the  disc,  hook  over  its  edge  in  a  manner 
characteristic  of  cilioretinal  \essels.  The  most  striking  feature  in  the 
funclus  is  a  vascular  loop,  twisted  like  a  rope,  which  projects  from  the  disc 
into  the  vitreous  for  a  distance  of  2.6  mm.  (8.D.).  It  originates  close  to 
the  central  vein  but  in  color  resembles  an  artery.  The  base  of  the  loop  is 
enclosed  in  connecti\e  tissue.  The  loop  undergoes  strong,  systolic  pulsa- 
tions during  which  its  anterior  extremity  rises  in  the  vitreous  and  the  entire 
coil  appears  to  elongate. 

Although  the  distribution  of  retinal  arteries  conforms  to  a  general 
plan,  the  widest  variations  in  their  arrangement  exist  upon  the  disc.  Fre- 
quently an  artery  and  vein  entwine,  a  vein  is  twisted  about  an  artery 
(Stergms.  11-33),  or  a  vessel  may  coil  upon  itself.  Vessels  may  leave 
the  ner\'e,  form  a  loop  and  re-enter  it  again.  Hirschberg  reported  a  case  in 
which  a  prepapillary  artery  entered  the  vitreous  where  it  broke  Into 
numerous  branches  which  entered  the  peripheral  parts  of  the  retina. 


30  THE    FUNDUS    OCULI. 

Diagnosis.  Preretinal  vessels  usually  are  confounded  with  the  per- 
sistent hyaloid  artery  from  fetal  life.  Examples  of  the  two  anomalies 
which  I  have  seen  present  essential  differences.  The  preretinal  artery  is  a 
well  formed  vessel  which,  after  its  excursion  into  the  vitreous,  returns  to 
the  nerve.  This  vessel  presents  every  appearance  of  having  always  pur- 
sued its  present  course.  The  hyaloid  artery,  on  the  contrary,  is  primarily 
distributed  to  the  fetal  lens  and  after  incomplete  absorption  it  does  not 
possess  the  finished  circulatory  connections  of  a  prepapillary  loop.  So- 
called,  persistent  hyaloid  arteries  consist  principally  of  membranous  rem- 
nants of  the  hvaloid  vascular  sheath,  the  artery  itself  frequently  being 
absent  or  forming  but  an  insignificant  part  of  the  structure. 

Stereogram  13.  Pseudoneuritis  and  Tortuous  Vessels  in 
Hypermetropia.  Right  eyeground  of  healthy  man,  27  years  of  age. 
Has  a  high  degree  of  hypermetropia  in  both  eyes  which  present  similar 
ophthalmoscopic  pictures.  With  correcting  glasses  the  vision  is  normal. 
The  fundus  is  darkly  stippled.  The  nerve-head  is  uniformly  reddened 
and  elevated  about  0.66  mm.  (  2.D. ) .  By  the  direct  method  of  examination 
the  margin  of  the  disc  is  indistinct  but  by  the  indirect  method  the  outlines 
become  sharp.  The  retinal  vessels,  both  arteries  and  veins,  are  very 
tortuous  but  the  arteries  are  not  disproportionately  narrower  than  the 
veins.  All  vessels  are  uniformly  broadened  and  present  neither  local 
constrictions  nor  ciilations.  There  are  no  hemorrhages  or  exudates  in  the 
retina.  Prolonged  observation  showed  the  fundus  condition  to  be  sta- 
tionary.    Diagnosis  is  hyperopic  fundus  or  pseudoneuritis. 

The  term  "  pseudoneuritis  "  is  applied  to  a  congenital  condition  which' 
beginners  in  ophthalmoscopy  almost  invariably  mistake  for  optic  neuritis. 
Nearlv  all  typical  examples  occur  in  highly  hyperopic  eyes,  i.  e.,  eyes, 
below  the  normal  size.  The  disc  is  dark  red  in  color  and  fine  super- 
numerary vessels  can  be  discerned  on  its  surface.  The  outline  of  the 
nerve  is  more  or  less  blurred  and  frequently  covered  with  delicate  striae, 
especially  on  the  nasal  side  where  nerve  fibers  are  most  abundant.  Some- 
times the  nerve  is  surrounded  by  a  delicate  grey  halo.  The  head  of 
the  nerve  may  project  1.-3.D.  thus  increasing  the  resemblance  to  papil- 
litis. The  disc  may  be  unusual  in  form  or  contain  an  inferior  crescent. 
The  retinal  vessels  are  unusually  tortuous  and  on  the  disc  they  may 
twist,  form  loops,  or  ciivide  in  an  erratic  manner.  The  appearance  of 
neuritis  presented  bv  highly  hyperopic  eyes  is  due  to  crowding  a  normal 
sized  retina  into  an  undersized  scleral  cavity.  As  a  result  the  retinal 
vessels  adapt  themselves  to   insufficient  space  by  becoming  tortuous,   the 


Fig.  oO. 
Coloboma  of  the  Optic  Nerve  and  Sheaths. 


i:'lg.   dl. 

Pseudoneuritis. 
Congenitallj-  small  eye.     Retina  crowded  into  an  undersized  Scleral   Cavity. 


CONCJENITAL    ANOMALIES.  31 

nerve  head,  o\ercrovvded  with  capillaries,  is  reddened,  and  the  ner\e  fibe'-'. 
unable  to  extend  their  full  length,  are  piled  up  on  the  disc.  In  hyperopia 
the  disc  is  redder  than  in  ernmetropia,  but  only  about  five  per  cent  of  cases 
exhibit  pronounced  pseudoneuritis.  Pseudoneuritis  is  a  stationary  con- 
dition, differing  in  this  respect  from  neuritis  which  either  recedes  or 
becomes  worse. 

In  all  the  congenitally  very  small  eyes  which  I  have  microscopically 
examined  the  retina  was  disproportionately  large.  The  exaggerated 
example  shown  in  Fig.  31  is  from  a  child  two  years  of  age.  Polar  diam- 
eter of  the  globe  was  18  mm. 

The  appearance  of  mild  neuritis  may  be  simulated  also  by  glassy, 
radiating  fibers  which  surround  the  disc  and  obscure  its  outline.  This 
picture  is  produced  bv  semiopaque  nerve  fibers  and,  if  careful  search  is 
made,  it  is  usual  to  find  a  few  densely  opaque  fibers  in  one  or  the  other 
eye.  A  deceptixe  appearance  of  commencing  edema  of  the  nerve  may  be 
produced  by  physiologic  light  reflexes.  Neither  of  the  two  last  mentioned 
conditions  is  accompanied  by  tortuous  vessels.  Slight  opacities  in  the 
cornea,  aqueous,  lens  or  vitreous  will  obscure  and  darken  the  disc  pro- 
ducing an  effect  frequently  mistaken  for  congestion. 

Dl'VGNOSIS.  Both  active  and  passive  hyperemia  of  the  disc  occur  but, 
owing  to  the  wide  \-ariations  in  color  presented  by  normal  discs,  the 
condition  is  extremely  difficult  to  diagnosticate.  Mere  redness  of  the 
nerve,  however  marked,  does  not  warrant  a  diagnosis  of  inflammatory 
hyperemia  unless  some  of  the  following  conditions  coexist: 

{(i)  Hyperemia  greater  than  previously  existed  in  the  affected  eye 
or  now  exists  in  the  fellow  eye. 

(h)    Redness  which   extends  into   a   physiologic  excavation. 

(r)  The  outline  of  the  disc  appears  blurred  by  both  direct  and 
indirect  methods  of  examination. 

(d)  Small  vessels  on  and  around  the  disc  increased  in  numbers. 

( e)  \'eins  enlarged  and  tortuous  with  arteries  straight  and  narrowed. 
(/)    Individual  vessels  exhibit  inequalities  in  breadth. 

(^)  Lymph  stasis  in  perivascular  sheaths  manifest  by  light  lines 
along  vessels,  on  and  near  the  disc. 

(h)    Absence  of  high  hypermetropia  and  astigmatism. 

True  neuritis  does  not  commence  with  diffuse  redness,  but  with 
distinct  edema  and  blurring  of  the  nasal  side  of  the  disc  and  adjacent 
retina  fStergm.  66).  As  stated  by  Frost,  congenitally  tortuous  vessels  do 
not  usually  rise  above  the  level  of  the  fundus,  while  in  pathologic  tortuosity 


32  THE    FUNDUS    OCULI. 

the  sinuosities  of  the  vessels  are  perpendicular  to  the  plane  of  the  retina. 
Pseudoneuritis  is  differentiated  from  true  neuritis,  chiefly,  by  absence  of  all 
pathologic  symptoms  and  presence  of  hypermetropia  or  astigmatism. 
Crowding  of  the  retina,  however,  is  not  due  to  refractive  error  but  to 
the  small  size  of  the  eye  and  small  eyes  are  not  always  hyperopic  or 
astigmatic. 

True  hyperemia  of  the  disc  may  accompany  choroiditis,  retinitis, 
diabetes,  nephritis,  high  blood  pressure,  polycythemia,  retrobulbar  neuritis, 
nasal  sinus  disease,  brain  lesions,  certain  diseases  of  the  central  nervous 
?v~>tem,  cerebral  sinus  thrombosis,  cardiac  disease,  mania,  etc.  It  may 
follow  poisoning  by  certain  drugs  especially  lead,   iodiform  and  alcohol. 

Strreogram  14.  Persistent  Hyaloid  Artery.  Persistent 
Canal  of  Cloquet.  Right  eyeground  of  a  girl  13  years  of  age.  Left 
eye  normal.  The  only  abnormality  observed  in  the  right  eye  is  a  bluish- 
white  cord  extending  from  the  center  of  the  disc  far  into  the  vitreous, 
terminating  in  a  number  of  filaments  (not  shown  in  picture).  Its  anterior 
extremity  is  seen  best  with  a  plus  lens  of  16.D.  The  slightest  movement 
of  the  eye  causes  active  oscillatory  motions  in  the  cord,  but  its  excursions  are 
of  limited  extent.  Xo  blood  vessels  are  visible  on  its  surface.  Evidently, 
the  formation  is  a  congenital  anomaly,  clinically  known  as  persistent 
hyaloid  artery. 

In  the  third  month  of  fetal  life  the  central  artery  of  the  retina, 
enclosed  in  a  cellular  sheath,  extends  through  the  vitreous  to  the  posterior 
surface  of  the  lens  which  it  invests  with  a  vascular  covering.  The  vessel, 
in  its  course  through  the  vitreous,  follows  the  hyaloid  (Cloquet's)  canal 
and  is  named  the  hyaloid  artery.  By  the  end  of  the  ninth  month  the  hyaloid 
artery  should  be  completely  absorbed.  Occasionally  the  artery  or  sheath 
is  not  entirely  removed,  in  which  case  they  persist  as  fetal  vestiges.  These 
remnants  of  intrauterine  life  show  the  widest  variations  in  structure. 
They  may  remain  attached  to  either  the  nerve,  the  lens  or  to  both.  The 
most  common  example  of  incomplete  absorption  is  an  opaque  patch  on 
the  posterior  capsule  of  the  lens  (posterior  polar  cataract)  which  may 
or  mav  not  be  accompanied  by  a  tag  of  tissue  on  the  nerve.  A  persistent 
hyaloid  artery  contains  tissues  from  both  the  artery  and  its  sheath.  When 
the  artery  persists  it  may  be  single  or  divided  into  several  branches.  A 
common  formation  is  a  cord  or  membrane  representing  either  the  ob- 
literated artery  or  the  cellular  sheath.  These  structures  vary  in  form 
from  a  thin  filamentous  thread  to  a  solid  column  of  connective  tissue  in 
which  blood  vessels  are  insignificant  or  absent.     Great  diversity  exists  in 


CONGENITAL    ANOMALIES.  33 

the  anterior  termination  of  the  cord  or  membrane;  thus,  it  may  be 
attached  to  the  lens,  divide  into  branches,  split  into  fine  filaments,  terminate 
in  a  bulb  or  in  a  sharp  point,  etc. 

Dlagnosis.  It  is  not  certain  that  all  the  membranous  and  vascular 
structures  in  the  vitreous,  clinicallv  known  as  persistent  hyaloid  arteries 
are  rightly  interpreted.  Eversbusch*  does  not  regard  differentiation  as 
easy,  and  separates  these  formations  into  two  classes:  (1)  True  per- 
sistent hyaloid^  artery;  (2)  Membranous  opacities  in  the  hyaloid  canal, 
probably  the  residuum  of  a  hemorrhage,  v.  Reusst  agrees  with  Eversbusch 
and  for  the  diagnosis  of  persistent  hyaloid  artery  requires  that  the  ves- 
sel should  distinctly  arise  from  the  arteria  centralis  retina?  and  be  filled 
with  blood.  Ciree^^t  diagnosticates  persistent  hvaloid  artery  when  the  cord 
is  connected  with  the  cei^tral  artery  and  posterior  pole  of  the  lens  or  else 
the  anterior  end  terminates  in  numerous  branches.  The  cord  sho'ld  not 
exceed  the  calibre  of  the  ce:-.tral  artery.  Greeff  further  diagnosticates  a 
visible  Cloquet's  canal  when  the  formation  has  a  funnel  or  ampullaform 
shape  anci  the  contents  are  opaque.  A  persistent  visible  canal  may  contain 
embryonic  tissue  or  a  well  developed  artery.  After  partial  absorption  of 
vitreous  exudates  I  h-ne  seen  opacities  persist  along  the  course  of  Clo- 
quet's canal  wb.ich  closely  resembled  persistent  fetal  elements.  Such  cases 
are  easily  differentiated  by  the  history  of  fluctuations  in  visual  power  which 
do  not  occur  with  fixed  congenital  defects.  The  membranes  of  retiriitis 
proliferans  usually  are  too  widely  distributed  to  be  mistaken  for  fetal 
remnants  of  the  hyaloid  canil.  Nevertheless,  after  injury  with  intraocular 
hemorrhage,  membranes  may  form  which,  in  the  absence  of  a  history,  could 
be  mistaken  for  persistent,  visible  hyaloid  canal  (Fig.  138).  A  pre- 
paj^illary  artery  (Stergm.  12)  is  frequently  miscalled  persistent  hvaloid. 
It  is  distinguished  by  its  completed  structure,  perfect  circulation  and  by  its 
origin  and  termination  in  the  nerve. 

Stereogram  15.  Albinis.nl  Eyeground  of  an  albino  girl  14  years 
of  age.  The  ophthalmoscopic  picture  is  that  of  an  exaggerated  blond 
fundus  in  which  the  normal  pigment  of  the  eye  is  absent.  The  background 
of  the  fundus  has  a  pinkish-white  color  produced  bv  the  white  sclera  shining 
through  the  capillary  layer  of  the  choroid.  The  optic  disc  Is  of  normal 
color  but  a-^pears  dirk  from  contrast  with  the  white  fundus.  The  blood 
vessels  form  a  beautiful  red  tracery  against  the  pink-white  sclera.  The 
retinal  vessels  lie  in   front  of  the  choroid   and   are   recognized  by  their 

*  EversViusch,  Mitt.  a.  d.  Klin.,   Miinchen,  1882. 

t  V.  Reuss,  Mitt.  d.  ii  Wien.  Klin.,  ii.  1885. 

t  Greeff,  Orth's  Sp.  Path.  Anat.  ii  Half,  ii,  p.  556. 

3 


34  THE    FUNDUS    OCULI. 

rounded  form  and  terminal  manner  of  distribution.  The  choroidal  vessels 
are  sharply  defined  against  the  light  background.  They  have  a  ribbon- 
like appearance,  possess  no  central  light  streak  and  anastomose  freely 
with  each  other.  The  deeper  red  color  of  the  macula  suggests  slight  pig- 
mentation of  that  region. 

Absence  of  the  physiologic  pigment  in  the  body  is  termed  albinism. 
The  anomaly  is  most  common  among  the  dark  skinned  races.  Albinism 
may  be  complete  or  incomplete.  It  would  appear  that  pigment  is  lacking 
in  the  eye  only  in  complete  cases,  and  it  is  not  uncommon  to  find  a  well 
pigmented  fundus  in  the  eye  of  an  individual  whose  hair  and  eyelashes 
are  nearly  albinotic.  Pigmentation  of  the  choroid  does  not  occur  until 
shortly  before,  or  even  after,  birth;  consequently,  infants'  eyes  frequently 
contain  islands  of  unpigmented  choroid,  particularly  at  the  periphery. 
Occasionally,  these  unpigmented  areas  persist  through  life.  Late  pig- 
mentation of  the  choroid  explains  why  some  cases  of  albinism  improve 
during  the  first  years  of  life.  In  complete  congenital  albinism  pigment 
has  not  been  deposited  in  the  skin,  hair,  eyelashes,  eyebrows,  uveal  tract 
or  retinal  epithelium.*  The  hair  is  fine  and  downy.  The  iris  is  a  light 
grey  color  but  becomes  pink  when  the  eye  is  transilluminated.  The  pupil 
has  a  deep  red  color:  It  is  usually  contracted  but  is  very  active.  The 
albinotic  iris  being  translucent,  fails  to  perform  its  normal  function  of  dia- 
phragm, to  camera,  and  these  unfortunates  are  almost  blinded  by  the 
excess  of  light  which  enters  the  eye.  In  their  efforts  to  escape  the  distress- 
ing illumination  the  eyelids  are  spasmodically  compressed  and  the  eyes 
rotate  from  side  to  side  until  permanent  nystagmus  is  established.  Myopia 
and  astigmatism  usually  develop,  probably  produced  by  pressure  and  trac- 
tion exerted  on  the  globe  by  spasm  of  the  extrinsic  muscles.  The  cells  of 
the  retina  and  choroid  are  normal  aside  from  absence  of  pigment.  Vision 
usually  is  subnormal,  but  the  color  sense  is  unaffected.  Naturally,  vision 
is  better  at  dusk  than  in  daylight.  No  cause  for  the  anomaly  is  known. 
Consanguinity  does  not  appear  to  be  influential  in  its  production.  It  is 
hereditary  and  may  occur  in  several  members  of  a  childship. 

Diagnosis.  Congenital  albinism  of  the  fundus  associated  with  gen- 
eral achromatism  is  almost  unmistakable.  Pathologic  depigmentation  of 
the  eyeground,  however,  does  occur  and  the  ophthalmoscopic  picture  which 

*  It  is  stated,  that  in  human  albinos  the  retinal  epithelium  always  contains  pig- 
ment. This  requires  confirmation,  inasmuch  as  no  pigment  is  contained  in  the  retinal 
epUhelium  of  albino  rabbits. 


CONGENITAL    ANOMALIES. 


35 


results  closely  resembles  that  of  the  albinotic  eye  (Stergm.  26).  I'he  only 
case  I  ever  saw  occurred  in  a  diabetic.  The  diiierential  points  of  this  case 
are  contained  in  the  following  table: 


CONGENLIAL   AlBLMS.M    OF 

Eyeground. 

Congenital. 

Associated  albinism  of  hair,  iris, 
etc. 

Photophobia;  under  proper  con- 
ditions vision  and  fields,  prac- 
tically,  normal. 

Vessels  of  choroid  normal. 

Depigmentation  of  fundus  uni- 
form and  complete. 

No  systemic  disease  present. 


Acquired    Albinism    of 
Eyeground. 

Acquired. 

Not  associated  with  general  al- 
binism. 

No  photophobia,  but  serious  im- 
pairment of  vision  and  fields. 

Many   choroidal    vessels    atrophic. 

Depigmentation  irregular.  Fun- 
dus contains  scattered  specs  of 
pigment. 

Affected  with  systemic  disease. 


Steri:o(;kam  16.  PuxcrATK  Condition  of  the  Fundus  (Gunn's 
Dots,  Crick  Dots).  Right  eyeground  of  a  healthy  housemaid  20  years  of 
age.  Fundus  conditions  are  identical  in  both  eyes.  With  correcting  glasses 
vision  is  normal.  I  he  macula  and  surrounding  region  contain  numerous 
groups  of  very  pale  dots,  situated  on  the  surface  of  the  retina.  The  nearer 
to  the  foN'ea,  the  more  distinct  are  the  dots. 

The  title  "  punctate  conditions  of  the  fundus  "  was  applied  by  Frost* 
to  a  group  of  varied  conditions,  some  of  which  have  since  been  more 
definitely  classified,  while  the  nature  of  others,  however,  remains  unknown. 
Chief  among  the  former  is  a  dotted  condition  of  the  macular  region,  first  de- 
scribed by  Marcus  Gunn  and  since  known  as  Gunn's  dots  or,  from  the  family 
name  of  Gunn's  first  cases,  "  Crick  dots."  These  terms  are  usually  applied 
to  small  groups  of  light  colored  spots,  situated  chiefly  at  the  fovea.  They 
appear  to  be  superficial  and  never  exceed  in  size  the  diameter  of  a  medium 
sized  retinal  vessel.  These  dots  are  inconspicuous  and  easily  overlooked. 
They  are  seen  best  by  weak  illumination  with  the  light  directed  obliquely. 
The  color  is  described  as  white  or  yellowish.  All  those  which  I  have  ob- 
served are  the  color  of  the  eyeground  but  very  much  paler.  Gunn's  dots 
occur  in  perfectly  normal  eyes  and  are  without  pathologic  significance. 
They  are  most  distinct  in  early  life  when  all  the  refracting  media  of  the 


*  Frost,  The  Fundus  Oculi.  1896. 


36  THE    FUNDUS    OCULI. 

eye  are  perfectly  transparent.  1  he  fundus  stereogram  here  shown  is  re- 
markable for  the  excessive  number  of  dots,  but  in  all  other  respects  the 
case  corresponds  with  Gunn's  description. 

The  anatomy  of  Gunn's  dots  is  unknown.  They  are  commonly  at- 
tributed to  inequalities  on  the  surface  of  the  retina  which  reflect  light.  In 
well  developed  cases  it  often  happens  that  the  retina  around  the  dots  is 
stippled,  an  appearance  presumably  due  to  rarefaction  of  the  epithelial 
pigment.  In  the  case  shown  in  the  stereogram  the  appearance  suggested 
that  the  dots  and  stippling  were  alike  produced  by  a  sieve-like  reticulation 
of  the  pigmented  epithelium. 

Diagnosis.  A  diagnosis  of  "  Gunn's  dots  "  should  be  made  only  in 
eyes  that  are  perfectly  normal;  never  where  deterioration  of  vision  has 
occurred.  Central  choroiditis  is  distinguished  by  the  greater  size  and 
distinctness  of  the  spots  and  by  visual  disturbance.  Colloid  excrescences 
(drusen)  usually  occur  in  the  aged.  They  are  more  widely  distributed 
and  more  distinct  than  Gunn's  dots.  Minute,  isolated  glistening  specks  are 
sometimes  seen  in  the  normal  fundus.  They  occur  at  all  ages  and  i-n  arty 
part  of  the  eyeground.  Their  brilliant  metallic  luster  is  sufficient  to  dif- 
ferentiate them. 


Fig.   8L'. 

Suppurative   Choroiditis. 

Retina  detached  Ly  Purulent  Exudates  from  Retina  and  Choroid. 


Fig.  33. 

Choroiditis. 

Stage  of  Exudation. 


Fig.    34 — -Choroiditis, 
Stage    of    Exudation. 


Fig.   34. 


Fig.    35— Choroiditis, 
Formation   of   Granula- 
tion    Tissue     which    in 
this      case      is      v  e  r  y 
'*'   ihundant. 


Fig.  35. 


Fig.    36 — Choroiditis    Stage    of    Cica- 
trization. 


i-lg.    oti. 


Fig.  oT — Choroiditis,  advanced  stage  of  Cica- 
trization.    Proliferation  of  Pigment  Cells. 


Fig.   38 — Choroiditis   Hyperplastica. 


Fig.  "38. 


Chapter  IV. 
AFFECTIONS  OF  THE  CHOROID. 

Inflammaticri  of  the  choroid  is  broadly  classed  as  suppurativj  (acute) 
and  exudative  (chronic)  Cic^roiditis.  Suppurative  choroiditis  is  an  acute 
infectious  process.  Oui.ijr  to  the  early  and  rapid  development  of  vitreous 
opacities  the  fundus  changes  cannot  be  seen  with  the  ophthalmoscope  ( Fig. 
32). 

Exudative  Choroiditis.  This  term  i:icludes  the  various  types  recog- 
nizable with  the  ophthalmoscope.  Inasmuch  as  the  retina  participates  in 
the  pathologic  process,  the  disease  is  also  ternied  chorioretinitis.  The 
clinical  forms  of  exudative  choroiditis  are  named  according  to  the  situa- 
tion, arrangement  or  form  of  the  lesions  as  follows:  c.centralis;  c.anterior; 
c.disseminata;  c.diflusa;  c.circinata;  c.areolaris;  c. guttata,  etc.  Many  of 
these  terms  are  merely  descriptive  of  special  cases,  not  special  forms  of 
disease.  The  majority  of  cases  present  scattered  foci  of  disease  which 
subsequently  may  fuse  and  form  large  patches.  A  diffuse  exudative  in- 
flammation of  the  choroid  is  uncommon. 

Pathology.  Individual  lesions  of  a  choroiditis  pass  through  three 
stages,  (1)  exudation  (Figs.  33-34)  ;  (3)  formation  of  granulation  tissue 
(Fig.  35);  (3)  cicatrization  (Figs.  36-37).  With  the  ophthalmoscope 
it  Is  not  practicable  to  differentiate  the  stage  of  exudation  from  that  of 
granulation.  In  the  exudative  stage  foci  of  round  cells  accumulate  about 
the  blood  vessels  of  the  inner  layers  of  the  choroid.  The  pigmented 
stroma  cells  of  the  choroid  emigrate  inward  and  appear  in  the  inner  layer 
where  they  are  never  found  under  normal  conditions  (Figs.  33-34).  At 
the  same  time  a  fluid  exudate,  which  varies  greatly  in  amount,  infiltrates  the 
choroid  and  separates  it  from  the  retina  (Fig.  34).  In  the  second  stage 
the  amount  of  granulation  tissue  varies.  Usually  it  is  slight  but  may  be 
abundant  (Fig.  35).  In  certain  cases  it  may  invade  the  vitreous  (cho- 
roiditis hyperplastica)  (Fig.  38).  The  retinal  epithelium,  which  is 
nourished  by  the  choroid,  being  deprived  of  nutrition  exfoliates  and  parts 
with  its  pigment  which  may  appear  on  the  surface  of  the  retina  in  the  form 
of  specks  or  branching  figures. 

Stereogram   17.     Choroiditis   Disseminata,   Exudative   Stage 

37 


38  THE    FUNDUS    OCULI. 

(Eruptive  Stage).  Right  fundus  oculi  of  a  woman  32  years  of  age. 
No  history  of  either  syphilis  or  tuberculosis.  Both  eyes  are  equally  affected. 
\'ision  20/40.  Flashes  of  light  and  a  "  glimmering  "  sensation  are  ex- 
perienced. The  ophthalmoscopic  picture  is  that  of  early  choroiditis  dis- 
seminata. The  fundus,  especially  the  equatorial  region,  is  covered  by  an 
eruption  of  discrete,  round,  softly-outlined,  reddish-yellow  spots,  situated 
below  the  level  of  the  retinal  vessels.  The  spots  vary  in  size  from  one- 
fourth  the  diameter  of  the  disc  to  that  of  a  primary  retinal  vessel.  At 
certain  points  they  occur  in  thickly  crowded  clusters.  In  this  early  stage  of 
the  disease  the  spots  show  but  slight  tendency  to  coalesce  and  their  borders 
are  not  pigmented.  Two  older  cicatricial  spots  are  seen,  bordered  by 
pigment.  Although  the  patient  is  a  brunette,  the  entire  fundus  is  light  in 
color  and  the  choroidal  vessels  are  plainly  seen,  indicating  slight,  general 
depigmentation  of  the  retinal  epithelium.  The  retina  is  transparent  and 
the  vessels'normal.  The  optic  disc  is  reddened,  presumably  due  to  hyper- 
emia of  the  choroid  which  has  extended  to  the  head  of  the  nerve  through 
the  anastomosing  vessels  of  Zinn. 

A  slight  knowledge  of  the  histologic  changes  described  enables  one 
to  interpret  the  picture  shown  in  the  stereogram.  For  example,  the 
flatteneci  leisons  and  transparent  retina  indicate  that  the  exudate  is 
slight  in  amount  and  the  granulation  tissue  not  exuberant.  The  color 
and  distinctness  of  the  spots  are  modified  by  the  amount  of  pig- 
ment remaining  in  the  overlying  retinal  epithelium;  thus,  where  the 
pigment  is  not  disturbed  the  lesion  is  obscure,  but  where  the  pigment  has 
been  partially  discharged  the  spots  are  grey,  while  those  spots  over 
which  depigmentation  is  complete  possess  a  reddish-yellow  color  produced 
by  the  red  choroid  seen  through  the  yellow  exudate.  Inflammatory  prod- 
ucts from  the  choroid  do  not  readily  pass  through  the  lamina  vitrea  into 
the  vitreous  unless  the  exudate  is  very  abundant  or  the  lamina  perforated. 
As  a  rule,  the  vitreous  is  clear  in  choroiditis  of  the  posterior  and  equatorial 
regions.  When,  however,  the  disease  develops  far  forward  or  involves 
the  ciliary  body,  the  anterior  part  of  the  vitreous  is  filled  with  floating 
opacities  (Fig.  39) . 

Exudative  choroiditis  is  caused  by  a  variety  of  general  infections, 
especially  syphilis  and  tuberculosis.  Other  causes  are  traumatism,  local 
mfection  and,  probably  intestinal  autointoxication.  In  many  cases  of  the 
disseminate  form  the  etiology  is  obscure.  The  course  of  disseminated 
choroiditis  is  slow.  Usually  the  prognosis  is  unfavorable.  In  certain 
cases,  however,  the  progress  of  the  disease  is  arrested,  but  the  diseased 


AFFECTIONS    OF    THK    CHOROID. 


39 


areas  cicatrize  and  remain  as  permanent  gaps  in  the  \isual  Held.  Choroidal 
lesions  at  the  macida  seriously  impair  vision,  but  situated  at  the  periphery 
they  may  not  be  troublesome. 

Diagnosis.  Choroiditis  in  the  exudative  stage  requires  ditterentia- 
tion  chiefly  from  angiopathic  retinitis  of  albuminuric  or  diabetic  origin. 
In  choroiditis  the  spots  are  yellowish  in  color,  deeply  situated  below  the 
level  of  the  retinal  vessels  and  some  pigmentation  is  usually  present.     In 


EXUDATIVE 

STAGE  OE 
CHOROIDITIS 
DTSSEMIN.\TA 

EXUD.ATIVE 

STAGE  OE 

AXGIOPATHIC 

ALBUMINURIC 

RETINITIS 

TYPICAL     CASES 

OF    CENTRAL 

PUNCTATE 

DIABETIC 

RETINITIS 

Number  and 

Situation 
of    Lesions. 

Numerous     and 
widelj'        distributed 
but   favor   e(iuatorial 
region.      .Always   be- 
low   level    of    retinal 
vessels. 

Comparatively  few 
in  number.    Favorite 
site    is   p  f)  s  t  c  r  i  o  r 
polar    zone.      Super- 
ficially   situated    and 
often    cover    the    re- 
tinal vessels. 

Whitish.  "  frosty  " 
color.     Circular  with 
no  tendency  to  coal- 
esce. 

Numerous.     Occur 
chiefly     in     macular 
region.    Superficially 
situated     and     cover 
retinal  vessels. 

Form  and 
Color. 

Yellowish  ;    circu- 
lar with  tendency  to 
coalesce     and     form 
scalloped    patches. 

Whitish,  round  and 
linear     spots     which 
do  not  coalesce. 

Pigmentation. 

.A.   few   pigmented, 
cicatricial    spots    us- 
ually present.    Fresh 
spots  may  be  "  pep- 
pered." 

No  disturbance  of 
pigment. 

No    pigmentation. 

Vessels. 

Retinal        arterio- 
sclerosis    forms     no 
part     of  '  choroiditis 
disseminata. 

.Arteriosclerosis 
usually   is   a   marked 
feature. 

Visible       vascular 
changes  are  unusual. 

Hemorrhage. 

No  retinal  hemor- 
rhage   in    uncompli- 
cated cases. 

Retinal       hemor- 
rhages usually  pres- 
ent. 

Numerous      small, 
retinal    hemorrhages 
between   spots. 

Optic   Nerve. 

Disc   may   be   red- 
dened   but    there    is 
no  neuritis.     Retinal 
edema     may    e.xtend 
to  nerve  (Stergm.  18). 

More  or  less  papil- 
litis usually  present. 

Neuritis  is  unusual 
in    this    form    of   re- 
tinitis.    Diabetic   re- 
tinitis frequently  in- 
distinguishable from 
other  forms  of  angio- 
pathic   disease. 

Retinitis  punctata  albescens,  senile  "  drusen  "  and  Gunn's  dots  are  noninflammatory 
punctate   conditions   not   apt   to   be   mistaken   for  any   of  the   above   affections. 


40  THE    FUNDUS    OCULI. 

retinitis,  on  the  contrary,  the  spots  have  a  whitish  "  frosty  "  color,  are 
superficially  situated,  often  overlying  the  retinal  vessels  and,  in  the  early 
stage,  are  never  accompanied  by  pigmentation.  The  lesions  of  choroid- 
itis are  numerous  and  widely  distributed  while  those  of  retinitis  are  com- 
paratively few  ill  number  and  usually  appear  around  the  posterior  pole. 
Vascular  degeneration,  retinal  edema  and  hemorrhage  which  belong  to 
the  picture  of  angiopathic  retinitis  are  exceptional  events  in  choroiditis. 
The  disc  may  be  reddened  in  choroiditis,  but  actual  neuritis,  so  common  in 
albuminuric  retinitis  does  not  usually  occur.  The  subjective  symptoms  of 
choroiditis,  i.  f.,  photopsia?,  metamorphopsias  and  chromctopsise  are  un- 
usual in  angiopathic  retinitis. 

Central  diabetic  retinitis  (Stergm.  38)  differs  from  choroiditis  in  the 
following  respects.  It  is  confined  chiefly  to  the  macular  region  and  is  ac- 
companied bv  hemorrhage;  the  spots  do  not  fuse  and  none  of  them  are 
pigmented  nor  do  they  undergo  cicatrization. 

Retinitis  punctata  albescens  (Stergm.  54)  is  easily  distinguished  by 
the  atrophic  condition  of  the  nerve  and  retina;  also,  by  the  subjective 
symptoms  of  night  blindness  and  contracted  fields. 

Stereogram  18.  Choroiditis  Disseminata,  Exudative  Stage. 
Right  eyeground  of  a  girl  15  years  of  age.  Hereditary  syphilitic.  Has 
saddle  nose,  notched  incisors,  scars  at  angles  of  mouth  and  arborescent 
corneal  opacities  characteristic  of  a  preceding  interstitial  keratitis.  Both 
eyes  affected.  The  left  eyeground  is  shown  in  stereogram  20.  Right 
Eye: — Central  vision  20/40.  Has  subjective  visual  sensations  consisting 
of  flashes  of  light,  balls  of  fire,  etc.  Unable  to  see  in  a  dim  light  (torpor 
retin-T).  The  entire  fundus  is  covered  with  spots  and  plaques  of  dis- 
seminated choroiditis.  Most  of  the  lesions  shown  in  the  picture  are  in 
the  stages  of  exudation  or  granulation,  but  those  in  the  anterior  zone  of  the 
fundus  (not  shown)  are  in  the  cicatricial  stage.  The  older  spots  point  to 
a  preceding  attack  of  choroiditis  which,  probably,  developed  in  connection 
with  a  keratitis  which  occurred  six  years  ago.  The  field  shown  in  the 
stereogram  is  covered  with  yellowish,  softly  outlined  spots.  Originally, 
all  the  spots  were  round,  but  many  of  them  have  coalesced  and  formed 
large  patches  with  scalloped  edges.  Some  of  the  spots  already  show 
commencing  marginal  pigmentation.  All  the  lesions  are  deeply  situated  and, 
when  in  contact  with  the  retinal  vessels,  the  latter  pass  over  them.  Two 
old,  well  pigmented  spots  are  seen  on  the  temporal  side.  On  the  nasal 
side  of  the  disc  is  a  large  circular  area  of  opaque  retina.  The  vessels  which 
curve  over  its  surface,  measured  with  the  ophthalmoscope,  are  about  0.5 


Fig.  39. 

Choroiditis. 

Vitreous  opacities  are  shown  in  the  upper  part  of  the  figure. 


Fig.  40. 

Choroiditis  causing  flat  Retinal  Detachment. 

An  exudation  from  the   choroid   at   E  has   lifted  and  infiltrated   the   retina. 


affec'tioxs  of  the  CIIORD'D.  41 

mm.  above  the  level  of  the  disc.  This  opaque  area  is  a  flat  retinal  detach- 
ment produced  by  a  recent,  profuse  exudation  from  the  choroid  which  has 
lifted  and  infiltrated  the  retina  (Fig.  40).  A  larger  amount  of  exudate 
would  have  passed  throuyjh  the  retina  and  appeared  in  the  vitreous.  The 
infiltration  of  the  retina  has  extended  along  the  nerve  fibers  to  the  disc 
and  obscured  its  outline  on  the  nasal  side.  I'his  condition  of  the  nerve-head 
is  distinguished  from  true  neuritis  by  its  evident  connection  with  adjacent 
edematous  retina,  also  by  the  absence  of  swelling  and  by  the  normal  retinal 
vessels.*  The  disease  is  a  wide-spread  intianmiation  of  the  choroid  mani- 
fest by  g'jncral  disturbance  of  choroid  and  retina  throughout  the  affected 
region.  In  many  places  the  surface  of  the  retina  contains  a  dust-like  pig- 
ment which  has  been  discharged  from  the  retinal  epithelium.  The  entire 
eyeground  is  mottled,  suggesting  foci  of  exudation  in  the  deep  layers  of  the 
choroid. 

SYMPTO^■s.  The  early  subjective  symptoms  of  exudative  choroiditis 
are  fairly  uniform  in  all  cases.  Photopsiae  occur  as  glimmering,  flashes  of 
light,  etc.,  due  to  disturbance  of  the  rods  and  cones  in  the  retina.  Another 
symptom  is  microps'ue,  i.  e.,  objects  appear  too  small.  This  phenomenon 
is  due  to  stretching  of  the  retina  and  separation  of  its  percipient  elements  at 
the  macula  by  exudate.  From  the  same  cause  objects  may  appear  distorted 
{melaniorphops'hc) .  In  other  cases  the  macular  structures  arc  crowded 
together  and  objects  appear  too  large  {  macro p she) .  \^isual  acuity  is 
always  blunted  in  disseminated  choroiditis,  but  the  loss  cannot  be  estimated 
from  the  ophthalmoscopic  picture.  Fair  central  vision  may  exi^t  in  con- 
nection with  wide-spread  destruction  of  the  fundus.  On  the  other  hand, 
vision  mav  be  greatly  reduced  with  but  slight  visible  change.  A  small 
central  lesion  mav  render  the  eye  useless,  while  a  large  peripheral* patch 
may  not  be  noticed  by  the  patient.  Torpor  retinae  usually  exists,  and  the 
patient  sees  well  only  in  bright  davlight  (hemeralopia) .  Exceptionally, 
the  retina  is  hypersensitive  and  vision  is  best  at  dusk  (nyctalopia).  There' 
may  be  acute  exacerbations  in  the  course  of  a  chronic  choroiditis.  In  the 
case  of  stereogram  18  a  preceding  attack  was  accompanied  by  interstitial 
keratitis,  the  choroiditis  being  confined  to  the  anterior  zone  of  the  fundus. 
Such  cases  are  common  in  congenital  syphilis.  The  keratitis  masks  the 
choroiditis  and  the  latter  is  not  discovered  until  the  cornea  clears. 

Diagnosis.  In  most  cases,  the  presence  of  old  choroidal  cicatrices 
renders  it  easy  to  recognize  the  primary  seat  of  the  disease.     In  rare  cases 


*  A  somewhat  similar  condition  of  the  disc  is  shown  in  stereogram  59. 


42 


THE    FUNDUS    OCULJ. 


chorioretinitis  commences  by  a  large  subretinal  effusion  which  produces 
an  opaque  patch  in  the  retina,  similar  to  that  seen  on  the  nasal  side  of  the 
disc  in  the  stereogram.  In  such  cases  the  ophthalmoscopic  picture  is  not 
unlike  that  which  follows  closure  of  a  branch  retinal  artery  (Stergm.  45). 
The  two  conditions  are  differentiated  as  follows: 


Opaque    Retina    Due    to 
Choroidal  Effusion. 

Surface  elevated. 

Outline   of  opacity  bears   no   rela- 
tion to  distribution  of  artery. 
Vessels  normal  or  dilated. 
Gradual  failure  of  vision. 
Photopsia?. 
Usually  some  pigment  disturbance. 


Opaque    Retina    Due   to 
Branch  Closure. 

Surface  not  elevated. 

Fan-shaped  opacity  corresponding 

to  distribution  of  branch  artery. 
Arteriosclerotic  changes  in  artery. 
Sudden   loss   of   vision   in   affected 

area. 
No  photopsia?. 
No  pigment  disturbance. 


Stereogram  19.  Central  Exudative  Choroiditis.  Exudative 
Stage.  (Syphilitic).  Right  eyeground  of  a  man  30  years  of  age.  Was- 
sermann  blood  reaction  for  syphilis  is  positive.  Acquired  chancre  ten 
months  ago.  Both  eyes  are  uniformly  affected.  Right  eye: — Vision  in 
full  daylight  is  20/70,  but  in  a  dimly  lighted  room  it  is  but  10/200. 
He  sees  flashes  of  light  and  shooting  stars.  Objects  at  which  he  looks 
appear  to  quiver.  The  anterior  part  of  the  vitreous  contains  fine,  dust-like 
opacities,  seen  best  by  weak  illumination  and  with  a  strong  convex  lens  in 
the  ophthalmoscope  (vitreous  opac'ties  not  shown  in  the  stereogram). 
Dust-like  vitreous  opacities  combined  with  choroiditis  are  regarded  as 
pathognomonic  of  svphilis.  The  optic  nerve  appears  reddened  and  its 
margin  blurred,  appearances  partlv  due  to  opacities  in  the  vitreous.* 
The  retinal  vessels  appear  normal.  The  macular  region  contains  a  group 
of  small,  rounded  spots  which  show  a  tendency  to  coalesce.  The  older 
spots  occupy  the  center  of  the  group.  They  are  whitish  in  color  and 
sprinkled  with  fine  pigment.  The  more  recent  spots,  situated  near  the 
periphery  of  the  group,  are  softly  outlined,  reddish-yellow  in  color  and 
nonpigmented.  The  normal  macular  light  reflexes  are  abolished,  due  to 
swelling  or  exudation  in  the  macular  depression. 


*  The  diagnosis  of  "  slight  neuritis,"  "  red  nerve."  etc.,  should  never  be  made  until 
it  is  certain  that  the  obscure  appearance  of  the  disc  is  not  an  artefact  caused  by  opaci- 
ties in  the  cornea,  aqueous,  lens  or  vitreous. 


AFFECTIONS    OF    THE    CHOROID.  43 

The  term  "  choroiditis  centralis  "  has  been  loosely  applied  to  several 
conditions  which  ditter  essentially  in  etiology  and  pathology.  These  are, 
(a)  choroiditis  of  syphilitic,  tubercular  or  uncertain  origin  (Stergm.  19- 
23)  ;  (h)  atrophy  and  degeneration  of  the  retina  and  choroid  in  myopia 
(Stergm.  71);  (c)  senile  changes  at  the  macula  (Stergm.  27);  (d) 
results  of  blunt  force,  hemorrhage,  etc.  (Stergm.  59);  (c)  congenital 
anomalies  f Stergm.  8). 

The  term  should  be  reserved  for  cases  included  in  class   (a). 

When  choroiditis  of  syphilitic  origin  appears  in  the  macular  region 
the  eruption  may  appear  as  a  large,  single  exudate  or  as  a  circumscribed 
group  of  closely  aggregated  spots  resembling  those  of  choroiditis  dis- 
seminata. The  symptoms  described  in  the  above  case  are  typical  of  the 
affection.  Ihe  subjective  symptoms  of  photopsiae,  quivering  of  objects, 
distortion,  etc.,  are  due  to  disturbance  of  the  cuter  retinal  layer.  The  an- 
terior, dust-like  \itreous  opacities,  usually  present  in  early  syphilitic  cases, 
come  either  from  the  anterior  extremity  of  the  choroid  or  posterior  ex- 
tremity of  the  ciliary  body.  If  the  ciliary  body  is  extensively  affected 
(cyclitis)  the  exudate  will  be  carried  forward  by  the  aqueous  current  and 
deposited,  in  the  form  of  fine  flecks,  on  the  lens  and  cornea.*  Exudates 
in  the  fore  part  of  the  \itreous  or  in  the  post  lental  region  may  be 
resorbed  with  astonishing  rapidity,  while  exudates  deep  in  the  vitreous 
disappear  slowly  and  often  incompletely. 

DiACNOSls.  Aside  from  positi\e  evidence  secured  by  the  history  and 
Wassermann  test,  syphilis  is  indicated  in  this  case  by  the  central  location 
of  the  eruption   and  vitreous  opacities. 

Colloid  excrescences  (drusen)  (Stergm.  27)  differ  from  a  central 
choroiditis  as  follows.  They  indicate  a  degenerative  process,  usually  senile, 
are  nonpigmented  and  cause  no  disturbance  of  vision. 

The  ophthalmoscopic  features  of  central  choroiditis  differ  from  Gunn's 
dots  (Stergm.  16)  in  the  following  respects.  The  choroidal  lesions  are 
more  distinct,  more  deeply  situated  and  attended  by  pigment  disturbance. 
Vision  is  affected  by  central  choroiditis  but  not  by  Gunn's  dots. 

Stereogram  20.  Choroiditis  DissexMixata,  Cicatricial  Stage. 
Left  eyeground  of  girl,    15   years  of  age.      (Right  eyeground  shown  in 


*  Cyclitic  deposits  in  the  anterior  chamber  were  long  misunderstood.     Hence  the 
misnomers   "  Descemitis,"   "  serous   iritis,"   etc.,    formerly   employed. 


44  THE    FUNDUS    OCULI. 

Stereogram  18).  Has  all  the  stigmata  of  congenital  syphilis,  i.  e.,  saddle 
nose,  notched  incisor  teeth,  cicatrices  at  angle  of  mouth  and  scars  of  an 
old  interstitial  keratitis.  Left  eye  possesses  only  bare  perception  of 
light.  Patient  is  uncertain  when  vision  was  lost  but  thinks  it  occurred 
during  an  attack  of  keratitis  six  years  ago.  The  vitreous  is  clear  as  is 
usual  in  late  choroiditis  disseminata.  The  entire  fundus  is  covered  with  the 
atrophic  lesions  of  exudative  choroiditis.  The  disease  has  run  its  course 
and  after  destruction  of  the  choroid  and  outer  layers  of  the  retina,  the 
havoc  has  been  repaired  by  formation  of  scar  tissue.  Complete  atrophy  of 
the  choroid  exists  in  the  broad,  white  zone  about  the  optic  disc,  where 
nothing  remains  of  the  vascular  structure.  Extending  putwards  from  the 
disc  into  the  macular  region,  is  a  broad,  dirty-brown  tract  which,  probably, 
marks  the  site  of  a  diffuse,  subretinal  exudation,  similar  to  that  existing 
in  the  left  eye  ( Stergm.  18).  Enormous  proliferation  of  pigment  has 
occurred  throughout  the  entire  fundus,  especially  around  the  cicatrices. 
The  active  proliferation  and  massing  of  pigment  at  the  border  of  a 
choroidal  cicatrix  mark  the  futile  efforts  of  the  pigmented  epithelium  to 
cover  over  the  defect.  Notwithstanding  extensive  destruction  of  the  cho- 
roid and  outer  layers  of  the  retina,  the  optic  nerve  and  retinal  vessels  are 
nearlv  normal,  indicating  that  the  inner  layers  of  the  retina  have  escaped 
atrophy.  The  outline  of  the  disc  is  blurred  due  to  absence  of  the  choroid 
which,  normally,  sharply  outlines  the  nerve.  Only  the  large  choroidal 
vessels  remain  In  the  atrophic  patches,  the  capillaries  and  vessels  of  medium 
size  having  been  destroyed. 

Repair.  Tn  exudative  choroiditis  repair  Is  effected  by  formation  of 
granulation  tissue  which  Is  replaced  by  a  fibrous  cicatrix  that  firmly  unites 
the  retina  and  choroid  (Fig.  41).  The  choroid  and  outer  layer  of  the 
retina  undergo  atrophy  and  form  a  thin,  translucent  membrane  (Fig.  42), 
through  which  the  white  sclera  Is  visible.  1  he  pigmented  cells  of  the 
choroid  (chromatophores)  usually  perish,  but  their  pigment  is  transported 
by  leucocytes  and  deposited  around  the  scar.  The  retinal  epithelium  over 
the  lesion  Is  destroyed,  but  the  surrounding  epithelial  cells  undergo  active 
proliferation,  covering  the  granulating  surface  and,  in  the  final  stage,  de- 
positing great  masses  of  pigment  on  anci  around  the  cicatrices  (Fig.  43). 
Exudates  or  hemorrhage  in  the  suprachoroldea  (Fig.  44)  frequently  or- 
ganize and  form  an  adventitious  membrane  outside  the  choroid  (Figs. 
45-46).  When  fully  oreanl^ed  these  suprachoroidcal  products  of  inflam- 
mation have  been  mistaken  for  fibromata   (Fig.  47).     The  structure  and 


Fig.  41. 

Choroiditis.  Stage  of  Repair. 

A  fibrous  cicatrix  at  a,  unites  the  retina  and  choroid. 


Fig.  42. 


Choroiditis,  Atrophic  Stage. 
R,  Retina;  S.  Sclera.     The  choroid  is  markedly  thin  and  atrophi' 


Fig.  43. 

Choroiditis,  Final  Stage  of  Atrophy. 

Masses  of  pigment  are  deposited  on  and  around  the  cicatrices. 


Fig.  44. 

Exudates  in  the  Suprach jro!dea. 

The   fibers  of  t'.ie   snprachoroidcT   nre   separated   and   distended   by   tlie   effusion,   shown 

ill   the   section  at   a. 


Fig.  45. 

Choroiditis,  Atrophic  Sta.q;e. 
Formation  of  advcntitidus  nu'inbranc  outside  the  choroid  fmm  or-Mnizalion  of  exudates 
and  heiiinrrliase.     M,  membrane;   B.  blood;   S.   -clc/a. 


Fig.  46.  Fig.   47. 

Fig.    46 — Choroiditis.      Atrophic    stage.  C.    choroid;    M,    adventitious    membrane; 

S,   sclera.      Fig.   47 — Choroiditis.      Organized  connective    tissue    of   the    suprachoroidea, 
resembling  fibromata. 


Fig.   4S. 

Choroiditis,  Stage  of  Repair.  -^ 

The  Choroid  is  partially  replaced  by  cicatricial   tissue.     The  eye  tension  has 

remained  normal. 


Fig.  49. 

Choroiditis,   Stage   of  Repair. 
Membranous  cicatrices  have  formed  in  the  Choroid. 


Fig.  51 — Formation  of  Flbrou'- 
Tissue   in   tlic   Choroid. 


Fig.  50 — Formation  of  irregular  masses 
of    Fibrous    Tissue   in   the    Choroid   of   a 

Plithisical   Eye. 


Fig.  51. 


Fig'.  52 — C'ssification  of  the  Choroid. 


Fir.  52. 


AFFECTIONS    OF    THK    CHOROID.  45 

amount  of  cicatricial  tissue  that  replaces  the  choroid  are  modified  by  in- 
traocular pressure.  If  the  eye  tension  remains  normal,  or  is  increased, 
the  cicatrix  is  membranous  or  composed  of  strands  of  connective  tissue 
(Figs.  48-49).  When,  however,  the  eye  softens  and  becomes  phthisical, 
thick,  irregular  masses  of  fibrous  tissue  develop  (  F"igs.  50-51).  These 
hypertrophic  fibrous  masses  mav  subsequently  be  converted  into  bone 
(Fig.  52). 

Unfortunately,  the  most  com-plcte  regeneration  attainable  after  cho- 
roiditis does  not  include  restoration  of  function,  and  every  developed  focus 
of  disease  becomes  a  permanent  blind  spot  in  the  field  of  vision.  The 
inner  layers  of  the  retina  may  or  may  not  atrophy.  Should  they  be  in- 
vaded by  the  disease,  secondary  optic  atrophy  results  and  the  disc  pre- 
sents the  characteristic  yellowish  tinge  of  retinitic  atrophy   (Stergm.  22). 

Diagnosis.  The  ophthalmoscopic  picture  (Stergm.  20)  is  typical  of 
choroiditis  disseminata  and  is  unlikely  to  be  mistaken  for  any  other  con- 
dition. When  choroiditis  disseminata  occurs  in  a  myopic  eye  it  is  apt 
to  be  overlooked  and  the  lesions  attributed  to  myopia.  In  such  cases,  the 
diagnosis  of  choroiditis  would  rest  upon  the  wide  distribution  of  the 
lesions,  excessi^•e  proliferation  of  pigment  and  eruptive  character  of  the 
disease.  Coloboma  of  the  macula,  senile  choroidal  atrophy,  posthemor- 
rhagic and  posttraumatic  cicatrices  form  single  or  local  lesions  while  the 
cicatrices  of  choroiditis  disseminata  are  distributed  throughout  the  fundus. 

Stereogram  21.  Central  Choroiditis,  Commencing  Cicatri- 
zation. Left  eyeground  of  a  man,  62  years  of  age.  Thirty-five  years  ago 
had  a  chancre  and  cutaneous  eruption.  Wassermann  reaction  for  syphilis,- 
positi\'e.  Two  vears  ago  had  persistent  headache  and  vomiting  which 
disappeared  under  antisyphilitic  treatment.  Six  months  ago  vision  became 
"foggy,"  and  since  then  it  has  steadily  deteriorated.  Present  vision  10/200 
in  each  eye.  Sees  best  in  a  dim  light,  scarcely  at  all  in  sunlight  (nyctalopia) . 
Small  central  scotomata  and  concentric  contraction  of  the  visual  fields. 
Argyl-Robertson  pupillary  reaction.  The  pathologic  changes  in  each 
eye  are  symmetrical.  The  central  temporal  quadrant  of  the  fundus  is 
riddled  with  small,  closely  aggregated  lesions  of  exudative  choroiditis.  In 
the  center  of  the  diseased  area  are  many  white  spots  encircled  by  pigment, 
which  represent  granulomata  that  have  been  converted  into  scar  tissue. 
Evidently,  the  disease  is  spreading  peripherally,  as  shown  by  groups  of 
recently  developed,  pink  colored,  nonpigmented  spots  in  the  outer  zone 


46  THE    FUNDUS    OCULI. 

of  disease.  Several  superficial  coal-black,  branching  figures  and  specks 
are  seen  in  front  of  the  spots.  These  are  formed  by  pigment  from  the 
tessellated  epithelium  which  has  been  carried  into  the  inner  layers  of  the 
retina.  The  optic  nerve  presents  the  picture  of  postinflammatory  atrophy. 
I'he  disc  is  white,  but  the  lamina  cribrosa  is  hidden,  the  retinal  vessels  are 
narrowed  and  the  nerve  is  surrounded  by  a  ring  ol  atrophic  choroid. 

Diagnosis.  The  symptoms  presented  by  this  case  are  complicated  but 
the  preceding  course  of  the  disease  may  be  conjectured.  Probably,  it  was 
as  follows.  Two  years  ago  intracranial  syphilis  with  vomiting,  headache 
and  choked  disc  which  subsided  under  antisyphilitic  treatment.  Later,  the 
constitutional  ciyscrasia  reappeared  as  central  choroiditis.  The  low  central 
vision  mav  be  attributed  to  choroiditis,  but  the  contracted  fields  and  nycta- 
lopia probably  depend  upon  disease  of  the  nerve  and  inner  retinal  layers. 
In  the  absence  of  all  history  the  ophthalmoscopic  diagnosis  would  lie 
between  central  choroiditis  and  senile  degeneration  with  colloid  excres- 
cences.    The  lesions  of  the  two  conditions,  however,  differ  as  follows : 

In  old  central  choroiditis  the  lesions  are  deeply  situated,  vary  in 
shape  and  size  and  tend  to  coalesce;  colloid  excrescences  are  very  super- 
ficiallv  situated,  uniform  in  size  and  do  not  coalesce.  In  choroiditis  the 
recent  spots  are  pink  in  color  and  old  spots  are  white  and  pigmented; 
colloids  are  uniformly  of  a  light  amber  color. 

Stereogram  22.  Chorioretinitis  Pigmentosa.  Retinitic  Optic 
Atrophy  (Yellow  Atrophy).  Left  fundus  oculi  of  a  boy  17  years  of 
age.  Father  contracted  syphilis  and  infected  mother  about  five  months 
before  the  patient's  birth.  Six  succeeding  children  were  all  born  dead. 
Patient  presents  the  stigmata  of  congenital  svphills.  Both  eves  affected 
with  chorioretinitis  but  in  varying  degrees.  Cannot  see  well  in  a  dim  light. 
Left  Eye: — Vision  reduced  to  mere  perception  of  light.  The  most 
conspicuous  ophthalmoscopic  feature  is  profuse  pigmentation  of  the  retina. 
The  pigment  is  situated  in  the  inner  layers,  as  shown  by  its  branching,  coral- 
like  forms  and  tendency  to  cover  the  retinal  vessels.  The  pigment  figures 
are  distributed  throughout  all  regions  of  the  retina  but  are  most  numerous 
at  the  equator  where  they  form  a  continuous  black  meshwork.  Pigment 
emigration,  in  this  fundus,  was  caused  by  diffuse  disease  of  the  inner 
layer  of  the  choroid.  The  outer  layer  of  large  choroidal  vessels  does  not 
appear  to  be  diffusely  affected.  In  certain  places,  however,  all  layers  of 
the   choroid   are  destroyed  and   replaced   by  white  cicatrices.      The   two 


AFFECTIOXS    OF    THE    CHOROID.  47 

lar^e  scars  are  composed  of  cicatricial  tissue  which  sends  prolongations  out 
over  the  surface  of  the  retina.  The  small,  round  hlack  spot  in  the  lower 
part  of  the  picture  mav  be  interpreted  as  a  cicatrix  in  which  the  retinal 
epithelium  has  succeeded  in  completely  covering  the  defect.  The  optic 
ner\e  has  the  dirty,  yellowish-white  color  which  characterizes  post  reti- 
nitic  atrophy.  The  retinal  vessels  are  uniformly  reduced  to  mere  threads. 
On  and  near  the  disc  the  vessel  walls  are  wliite.  I  he  lamina  cribrosa 
does  not  appear  and  the  entire  disc  appears  obscured  as  by  a  thin  \eil. 

The  layer  of  pigmented,  tessellated  epithelium  which  lines  the  inner 
siirface  of  the  choroid  is  known  as  retinal  epithelium  because  it  develops 
from  the  same  blastocbrmic  layer  as  the  retina.  Anatomicilly  and  patho- 
logically, however,  it  is  a  part  of  the  choroid  and  participates  in  its  diseases. 
That  the  epithelial  lav:r  may  be  a  primary  seat  of  disease  is  extremely 
doubtful,  iiiasmuch  a,^  it  contains  no  blood  vessels  and  is  dependent  upon 
the  choroid  for  nutrition.  The  indi\Mdual  epithelial  cells  appear  to  pos- 
sess migratorv  powers  and  when  deprixed  of  nutrition,  through  choroidal 
disease,  they  may  wander  oiit  into  the  retina.  These  wandering  cells 
either  lose  their  pigment  and  perish  or  form  attachments  and  proliferate 
(Fig.  37).  In  diffuse  choroiditis  not  attended  by  profuse  exudation,  pig- 
ment discharged  from  the  retinal  epithelium  is  transported  bv  leucocytes  or 
lymph  currents  and  deposited  around  the  arterioles  and  capillaries  of  the 
retina,  where  it  inrms  hranchin:';  lines  and  figures  the  ophthalmoscopic  ap- 
pearance of  which  is  aptly  compared  to  bone  corpuscles  or  Haxersian  canal 
systems. 

This  fundus  exhibits  a  few  cicatrices  of  deep,  focal  choroiditis,  and 
also  unixersal  disturbance  of  the  retinal  epithelium  indicating  diffuse  dis- 
ease of  the  choriocapillaris.  Choroidal  disease  does  not  usually  affect  the 
inner  lavers  of  the  retina,  but  in  this  eye  these  layers  are  highly  atrophic 
as  manifested  by  pigmentation,  extremely  narrow  blood  vessels  and  yellow 
atrophy  of  the  nerve.  As  the  nutrition  of  the  inner  layers  is  independent 
of  the  choroid,  their  ciestruction  depends  on  either  inflammation  of  both 
choroid  and  retina  due  to  action  of  a  common  toxin  or,  what  is  more 
probable,  inanition  of  the  retinal  tissue  due  to  blocking  of  the  lymph  chan- 
nels with  pigment. 

Diagnosis.  This  affection  is  frequentlv  mistaken  for  retinitis  pig- 
mentosa. In  this  particular  case  the  presence  of  choroidal  cicatrices  would 
prevent  such  an  error.     Frequently,  however,  the  resemblance  is  so  close 


48 


THE    FUNDUS    OCULI. 


that  differentiation  is  extremely  difficult.  The  following  differential  points 
are  applicable  to  the  majority  of  doubtful  cases.  The  blood  reactions 
for  syphilis  should  also  be  employed. 


Chorioretinitis   Pigmentosa. 

May  commence  at  any  age. 

Not  a  family  disease. 

Nirrht  blindness  appears  in  ad- 
vanced cases. 

May  commence  in  any  part  of  the 
fundus. 

May  attack  but  one  eye. 

Development  and  progress  may 
vary  in  the  two  eyes. 

Central  vision  usually  affected 
early. 

D'lipent  search  usually  reveals 
white  scars  in  funcius. 

Irregular  distribution  of  pigment 

Only  physical  defects  are  stigmat:i 
in  cases  due  to  syphilis. 

Disease  may  be  arrested. 

If  central  or  scattered  scotomata 
can  be  demonstrated  it  is  not  a 
case  of  retinitis  pigmentosa. 


Retinitis  Pigmentosa 
(Stergm.  53) . 

Commences  in  infancy  or  early 
childhood. 

Reappears  in  a  family  for  genera- 
tions. 

Xight  blindness  invariably  present 
from  onset  of  disease  and  pre- 
cedes pigmentation. 

Commences  anterior  to  equator  and 
ad\-ances  slowly  backward. 
Reaches  macula  in  adult  life 
(30-50). 

Invariably,   affects  both   eyes. 

De\'elops  simultaneously  and  pro- 
curesses with  great  uniformity  in 
both  eyes. 

Central  vision  usually  retained  until 
last  stage  of  disease. 

Choroidal  cicatrices  never  present 
In  uncomplicated  cases. 

Pigment  uniformly  distributed. 

Large  percentage  of  cases  associ- 
ated with  body  malformations, 
idiocy,  or  deafmutism. 

Progress  of  disease  never  arrested. 

The  typical  visual  field  is  concentric 
contraction  which  commences  as 
ring  scotoma. 


Stereogram  23.  TT'BERculosis  of  the  Choroid.  Right  eyeground 
of  a  man  21  years  of  age.  A  sister  has  pulmonary  tuberculosis.  Left  eye 
normal.  In  the  right  eye  disturbance  of  \ision  commenced  two  months 
before  coming  under  observation — at  which  time  it  was  reduced  to  hand 
movements.     \'!treous  was  cloudy  and  filled  with  floating  opacities.     With 


AFFECTIONS    OF    TUl.    C[:C)R:).D.  49 

the  ophthalmoscope  the  fundus  appeared  dark,  except  a  large  area  on  the 
temporal  side  which  gave  a  white  reflex  like  a  hright  light  seen  through 
dense  fog.  No  fundus  details  discernible.  Skin  test  for  tuberculosis 
positive.  Under  full  injections  of  tuberculin  there  was  sudden  aggra- 
vation of  the  fundus  conditions  accompanied  by  intense  headache,  hacking 
cough  and  fever.  The  increased  eye  disturbance  was  regarded  as  a  local 
reaction  to  tuberculin  and  positive  evidence  that  tubercular  choroiditis  was 
present.  After  subsidence  of  the  reaction,  tuberculin  was  resumed  in  less- 
ened doses.  Improvement  followed,  terminating  in  recovery  with  central 
vision  of  20^30  but  with  an  absolute  scotoma  corresponding  to  the  cho- 
roidal lesion.* 

The  stereogram  was  made  four  months  after  treatment  was  begun. 
The  V'itreous  opacities  have  disappeared.  The  disc  is  reddened.  Retinal 
vessels  normal.  Just  beyond  and  slightly  above  the  macula  is  a  round, 
yellowish-white  patch  with  a  diameter  about  three  times  that  of  the  disc. 
It  is  surrounded  by  a  number  of  similar  but  much  smaller  spf)ts.  All  ether 
parts  of  the  fundus  are  free  from  disease.  The  lesions  described  are 
supposed  to  be  a  herd  of  tubercles.  The  large  patch  has  a  well  defined, 
soft  outline.  The  retinal  x'essels  which  curve  over  its  surface  are  elevated 
0.5  mm.  above  the  normal  level  of  the  fundus.  The  surface  of  the  patch  is 
entirely  depigmented  but  along  its  inner  border  pigment  has  emigrated  and 
settled  around  the  descending  macular  vein.  Beyond  the  outer  horde*-  of 
the  patch  the  retinal  pigment  is  thinned,  exposing  the  choroidal  vessels. 
The  smaller  deposits  resemble  the  large  one. 

Tuberculosis  has  long  been  regarded  as  a  probable  cause  of  obscure 
cases  ot  choroiditis,  but  only  since  the  employment  of  tuberculin  in  diag- 
nosis has  it  been  demonstrated  that  the  proportionate  number  of  tuber- 
culous cases  is  large.  Formerly,  but  two  types  of  tuberculosis  were  recog- 
nized in  the  choroid,  i.  e.,  miliary  tubercle  which  appears  in  the  late  stage 
of  general  miliary  tuberculosis,  and  conglomerate  tubercle  which  forms  an 
intraocular  tumor.  At  the  present  time  it  is  believed  that  tuberculosis  may 
appear  also  as  an  exudative  choroiditis,  focal  or  diffuse,  and  that  the  oph- 
thalmoscopic picture  is  by  no  means  constant.  Pure  tubercle  is  a  non- 
inflammatory, nonvascular  growth  which  replaces  the  tissue  in  which  it 
grows ;  facts  to  be  borne  in  mind  when  diagnosticating  this  disease.  Authors 
have  remarked  the  absence  of  pigmentation  in  early  tubercular  lesions  of 
the  fundus.     Conclusive  observations  upon  this  point  are  lacking,  but  it 


*  Patient  of  Dr.  \Vm.  G.  Reynolds. 
4 


50  THE    FUNDUS    OCULI. 

is  probable  that  the  chromophores  of  the  choroid  and  retinal  epithelium  are 
completely  destroyed  and  replaced  by  tubercle.  When,  however,  a  tuber- 
cular lesion  of  the  choroid  heals,  the  surrounding  epithelium  proliferates 
and  endeavors  to  repair  the  damage  as  in  loss  of  tissue  from  other  causes, 
consequently,  a  posttubercular  cicatrix  is  pigmented.  The  amount  of  dis- 
turbance created  in  adjacent,tissues  by  intraocular  tubercle  appears  to  vary 
according  to  the  location  of  the  deposit.  In  the  ciliary  body  T  have  seen 
tubercle  perforate  the  sclera  with  little  or  no  intraocular  disturbance  (Fig. 
225).  In  the  choroid,  tubercle  usually  produces  edema  or  flat  detachment 
of  the  retina  and  cloudiness  of  the  vitreous.  Nevertheless,  from  the  known 
nature  of  tubercle,  it  is  probable  that  the  disturbance  is  mechanical  rather 
than  inflammatory.  All  tissues  crumble  before  the  advance  of  tubercle; 
thus,  destruction  of  the  lamina  vitrea  permits  fluid  to  pass  freely  through 
the  retina  into  the  vitreous  (Fig.  S3).  The  retina  rnay  be  slightly  elevated 
by  fluid,  but  usually  is  invaded  before  detachment  occurs.  When  tubercle 
invades  and  closes  the  retinal  vessels,  hemorrhages  may  ensue.  Axenfeld 
and  Stock  hold  that  there  may  be  tuberculous  disease  of  intraocular  blood 
vessels  without  visible  fundus  changes.  Miliary  tubercles  appear  to  create 
little  local  disturbance.  Quiescent  tubercle  of  the  choroid  is  prone  to 
relapse;  thus,  an  old  cicatrix,  in  which  tubercle  bacilli  are  enclosed,  may 
break  down  and  become  active.  It  is  only  tubercular  scars  that  act  thus 
as  a  nidus  of  disease  in  the  choroid.  Tuberculosis  affects  the  choroid 
in  connection  with  remote' parts,  although  it  does  not  appear  possible  for 
an  intraocular  deposit  to  be  the  only  tubercular  focus  in  the  body.  Certain 
forms  of  tuberculosis  haxe  a  tendency  to  deposit  metastases  in  the  choroid, 
i.  e.,  tubercular  meningitis  of  children  and  general  miliary  tuberculosis. 
On  the  other  hand,  the  choroid  is  seldom  affected  in  pulmonary  tuberculosis. 
Miliary  tubercles  appear  in  the  choroid  as  very  small,  softly  outlined  spots, 
at  first  grey  but  later  yellowish  in  color.  They  rapidly  increase  in  size  but 
do  not  exceed  1/3  disc  diameter  (Colored  Plate  2).  The  presence  of 
miliary  tubercles  in  the  choroid  is  of  great  importance  in  diagnosticating 
doubtful  cases  of  continued  fe\'er. 

Pathologists  recognize  three  forms  In  which  tuberculosis  occurs  in 
the  choroid:  (1)  Miliary  tubercle,  present  in  nearly  all  cases  of  general, 
miliary  tuberculosis.  Miliary  tubercles  are  most  frequent  around  the  disc 
but  are  found  In  all  parts  of  the  choroid.  They  are  most  frequently  found 
In  the  outer  layers  where  they  project  outwards  Into  the  suprachoroidea, 
but  they  may  be  deposited  in  all  layers.  As  a  rule,  they  are  discrete 
although  fusion  may  occur.     The  developed  nodules  exhibit  the  structure 


Fig.  53. 

Tuberculosis  of  the  Clioroid. 

Tubercle,  shown  at  T  in  the  section.     The  retina  over  it  is  elevated  by  fluid. 


Fig.  o-l. 
Typical   Miliary  Tubercle. 
^Specimen    from   the    Iris.'> 


AFFECTIONS    OF    THE    CHOROID.  51 

typical  of  miliary  tubercle,  consisting  of  the  Langhans  type  of  giant  cell 
surrounded  by  epthelioid  and  mononuclear  cells  (Fig.  54).  Tubercle 
bacilli  may  or  may  not  be  demonstrable  in  the  tissues.  (2)  Diffuse  uveal 
tuberculosis.  The  entire  uvea  is  involved.  The  choroid  may  be  uniformly 
thickened  by  development  of  granulation  tissue,  containing  giant  cells  and 
poorly  developed  tubercle  systems.  The  advancing  edge  of  the  diffuse 
type  exhibits  tubercular  nodules.  Fhe  disease  may  terminate  by  perfora- 
tion of  the  sclera  or  the  granulation  tissue  may  organize  into  scar  tissue 
as  occurs  in  exudative  choroiditis.  (.3)  Solitary  or  conglomerate  tubercle 
which  occurs  in  any  part  of  the  u\'eal  tract.  When  fully  developed  it 
forms  an  intraocular  tumor  which  may  closely  resemble  gliomas  retinae. 
There  is  no  doubt  that  single  tubercles  of  large  size  develop  in  the  choroid 
and  subsequently  undergo  cicatrization. 

Diy\GNOSis.  MiLE'XRY  TuBERClfLOSls.  The  best  evidence  that  recent, 
choroiditic  spots  are  tubercular  is  a  coexisting  general  miliary  tubercu- 
losis or  tubercular  meningitis.  On  the  other  hand,  it  occasionally  haupens 
that  the  nature  of  an  obscure  fever  or  cerebral  disease  is  determined  o  ilv 
by  discovery  of  miliary  tubercles  in  the  choroid.*  As  distinguished  from 
choroiditis  disseminata,  miliary  tubercles  appear  in  the  choroid  as  small, 
points  which  grow  very  rapidly  until  they  attain  a  maximum  size  of  about 
1/3  disc  diameter.  The  spots  of  choroiditis,  on  the  contrarv,  change  verv 
slowly.  Miliary  tubercles  are  nonpigmented,  while  choroiditis  is  atter.d^d 
by  more  or  less  pigme-^tition.  I'he  constitutional  disturbance  of  miliarv 
tuberculosis  is  absent  in  choroiditis. 

CoNGLOMHRATE  TuiJERCLE  (tuberculoma)  of  the  choroid  occurs 
almost  exclusively  in  the  young.  Occurring  in  those  under  five  years  of 
'age  it  requires  to  be  differentiated  from  glioma  of  the  retina.  Both  con- 
ditions are  rare.  Positive  local  reaction  to  an  injection  of  tuberculin 
would  be  conclusive  evidence  that  the  tumor  was  tubercular.  Cases  are 
reported  of  tuberculoma  with  extensive  detachment  of  the  retina,  ophthal- 
moscopically  indistinguishable  from  glioma.  Glioma  is  said  to  cast  no 
shadow  in  transillumination  of  the  eye.  Theoretically,  tuberculoma  of  the 
choroid  should  cast  some  shadow  in  transillumination  since  it  grows  in 
intimate  contact  with  the  sclera,  which  is  not  the  case  in  glioma.  When 
conglomerate  tubercle  of  the  choroid  is  oDhthalmoscopically  visible. 
It  appears  as  a  light  colored  tumor  accompanied  by  neighboring,  smaller 
nodules.      The   presence    of   these    "  daughter"    deposits    is    regarded    as 


*  Tuberculin   usually  fails  as  a  means   of  diagnosis  in  the  late   stages  of  general 
tubercular  infection. 


52 


THE    FUNDUS    OCULI. 


Strong  evidence  that  a  tumor  is  tubercular.  Tubercle  is  a  nonvascular 
growth,  consequently,  if  adventitious  blood  vessels  are  seen  on  the  surface 
of  a  tumor,  they  point  to  glioma  or  sarcoma. 

Septic  Retinitis  of  Roth  is  a  punctate  condition  of  the  fundus  which 
raav  appear  in  the  late  stage  of  sepsis.  Ihe  spots  are  whiter  than 
tubercles  and  usually  accompanied  by  hemorrhages.  In  the  absence  of 
historv,  however,  differentiation  from  miliary  tuberculosis  would  be 
extremely  difficult. 

Focal  Tuberculosis  of  the  Choroid.  Cases  of  tubercular  patches 
in  the  choroid  which  have  been  studied  with  the  ophthalmoscope  are  so 
few  in  number  that  a  characteristic  fundus  picture  of  the  condition  has 
not,  as  vet,  been  formulated.  Nevertheless  this  is  the  most  common  type 
of  intraocular  tuberculosis.  It  appears  in  all  parts  of  the  uveal  tract. 
Choroiclitis  in  children  and  young  adults  is,  in  the  absence  of  syphilis, 
presumably  tuberculous.  The  best  evidence  that  a  choroiditis  is  tuber- 
cular is  local  activity  in  the  diseased  area  following  injections  of  tuberculin. 
In  all  doubtful  cases  the  blood  reactions  for  syphilis  should  also  be 
employed. 

The  following  table  will  indicate  the  chief  points  of  ciistinction : 


Focal  Tubercular  Choroidi:  is. 

Frequently  unilateral  usually  con- 
fined to  a  limited  area. 

(May  spread  throughout  fundus). 

Extends  by  development  of 
"  daughter  "  tubercles  in  prox- 
imity to  original  tubercle. 

Old  cicatrices  may  be  starting 
point  of  a  fresh  outbreak. 

Surface  usually  more  deviated  than 
in  exudative  choroiditis. 


Exudative  Choroiditis. 


Usually  bilateral. 

Widely  disseminated. 

Appears  simultaneously  throughout 

wide  areas. 
Cicatrices     once     formed     remain^ 

quiet. 
Surface  not  elevated  except  in  cases 

of      subretlnal      effusions      and 

edema. 


Stereogram  24.  Diffuse  Choriorftinhis,  Atrophic  Stage. 
Rftinttic  Optic  Atrophy  (Yellow  Atrophy).  left  eyeground  of  a 
man  41  years  of  age.  No  history  or  symptoms  of  syphilis.  Wassermann 
reaction  negative.  Several  immediate  relatives  have  been  affected  with 
tuberculosis.  The  right  eye  has  normal  vision  and  the  fundus  presents 
no  evidence  of  disease.  In  early  manhood  painless  loss  of  vision  occurred 
in  the   left   eve.      Central   vision    is    20  TOO.      The    visual    field   contains 


AFFIXTIONS    OF    THE    CHOROID.  53 

large  irregular  defects  which  correspond  to  areas  of  destruction  in  the 
choroid.  7'he  ophthalmoscopic  picture  is  that  of  wide-spread  vascular 
degeneration  of  the  choroid,  and  moderate  atrophy  of  the  retina.  Ex- 
cept in  the  macular  region,  the  retinal  epithelium  has  lost  its  pigment, 
thereby  exposing  the  vessels  of  the  choroid.  A  few  masses  of  this  pigment 
are  scattered  throughout  the  retina.  The  disc  is  surrounded  by  a  broad, 
white  area  in  which  the  choroid  has  undergone  extreme  atrophy. 

It  is  noticeable  that  in  nearly  all  cases  of  wide-spread  atrophy  of 
the  choroid  the  process  is  furthest  advanced  around  the  nerve.  1  he 
reason  for  this  is  not  altogether  clear,  although  it  may  be  due  to  the 
close  adhesion  between  choroid  and  sclera  that  normally  exists  in  this 
region,  while  elsewhere  the  two  membranes  are  separated  by  lymph  spaces. 
The  retina  is  somewhat  atrophic  as  shown  by  pigmentation,  slight  narrow- 
ing of  the  retinal  vessels  and,  especially,  by  yellow  atrophy  of  the  nerve. 
On  the  outer  side  of  the  macula  is  a  large  atrophic  patch  in  which  only 
a  few  of  the  larger  choroidal  vessels  remain.  Similar  patches  exist  in 
the  periphery  of  the  fundus  which  do  not  appear  in  the  picture.  A 
sclerosed  choroidal  vessel  may  appear  like  a  solid,  white  band,  never- 
theless, it  mav  contain  a  lumen  through  which  blood  circulates.  This 
choroid  has  passed  through  a  diffuse  inflammation  of  sufficient  intensity 
to  cause  irregular  sclerosis  of  the  vessels.  In  those  regions  of  the  fundus 
presenting  a  red  reflex  the  retinal  epithelium  has  not  been  entirely 
dispersed,  and  the  dark  intervascular  spaces  of  the  choroid  indicate 
the  presence  of  choroidal  pigment.  The  dirty-yellowish  patches  around 
the  disc  and  elsewhere,  mark  the  site  of  an  intense  inflammatory  process 
which  has  destroyed  the  pigmented  cells  and  vessels  of  the  choroid.  After 
inflammation  of  the  choroid  has  subsided  the  membrane  may  pass  through 
the  varied  stages  of  tissue  degeneration  including  the  deposit  of  cal- 
careous matter  and  formation  of  bone.  These  advanced  degenerative 
processes  usually  occur  in  soft  eyes  although  bone  may  form  in  a  sclerosed 
area  around  the  disc  even  though  eye  tension  is  not  lowered. 

The  cause  of  choroiditis  in  this  eye  was  not  ascertained.  Whatever 
the  toxin,  It  was  no  longer  activ^e  when  the  patient  came  under  observation. 
Syphilitic  choroiditis  usually,  but  not  invariably,  affects  both  eyes.  Oc- 
ular tuberculosis,  on  the  other  hand,  generally  is  unilateral  and  it  is  not 
improbable  that  this  is  a  picture  of  healed  tuberculous  lesions.  I  once  saw  a 
similar  eyeground  in  a  young  woman,  in  which  tuberculosis  developed  in  an 


54  THE    FUNDUS    OCULI. 

old  cicatricial  patch.  This  form  of  choroiditis  differs  from  choroiditis  dis- 
seminata in  being  unilateral  and  non-progressive,  also,  by  its  patchy, 
irregular  distribution. 

Stereogram  25.  Vascular  Degeneration  of  the  Choroid 
(Sclerosis  of  the  Choroid).  Atrophy  of  Retina  and  Nerve.  Right 
eyeground  of  man  32  years  of  age.  Six  years  ago  a  violent  blow  on  the 
right  brow  produced  complete  and  permanent  blindness.  It  is  probable 
that  an  orbital  fracture  occurred  which  extended  through  the  optic  canal 
and  lacerated  the  optic  nerve.  The  same  injury,  i:i  some  way,  closed 
the  ophthalmic  artery,  thereby  interrupting  the  blood  supply  to  the  cho- 
roid and  retina.  The  ophthalmoscopic  picture  is  the  same  as  follows 
optico-ciliary  neurectomy.  The  vessels  of  the  choroid  are  highly  sclerosed 
but  not  entirely  bloodless.  Degeneration  is  furthest  advanced  around  the 
disc  where  the  vessels  are  converted  into  white  strands  of  connective 
tissue.  At  the  equator  the  fundus  presents  a  red  reflex.  The  retinal 
epithelium  has  parted  with  much  of  its  pigment  some  of  which  has  emi- 
grated and  formed  branching,  spider-shaped  figures  in  the  retina.  The 
inner  lavers  of  the  retina  are  known  to  be  highly  atrophic  by  the  extreme 
narrowing  of  the  main  vessels  and  disappearance  of  all  small  branches. 
The  optic  nerve  is  very  atrophic,  sharply  outlined  and  slightly  excavated. 
It  is  unusual  to  see  complete  excavation  of  the  disc  in  a  nonglaucomatous 
eye,  but  in  this  case  it  may  legitimately  be  due  to  extreme  atrophy  and  con- 
traction of  the  nerve.  The  same  condition  is  occasionally  found  in  micro- 
scopic examinations  of  atrophic  bulbs  in  which  the  retina  is  not  detached 
(Fig.  ')5).  In  the  eye  shown  in  the  stereogram  the  tension  is  normal,  the 
media  clear  and  there  is  no  evidence  of  iritis  or  other  inflammation;  there- 
fore, the  fundus  changes  may  be  attributed  to  malnutritive  degeneration  of 
the  choroid  and  retina.  This  interpretation  of  the  ophthalmoscopic  picture 
is  based  on  the  results  of  animal  experimentation.  Thus,  Wagenmann  cut 
the  ciliary  arteries  which  supply  the  choroid  without  injuring  the  retinal 
vessels.  The  result  was  degeneration  of  the  choroid,  destruction  of  the 
retinal  epithelium,  pigmentation  of  the  retina  and  degeneration  of  its  outer 
layers.  On  the  other  hand,  section  of  only  the  optic  nerve  and  retinal 
vessels  without  disturbing  the  ciliary  arteries,  resulted  in  degeneration  of 
the  inner  retinal  layers  but  there  was  no  degeneration  of  the  choroid  or 
pigmentation  of  the  retina.  FVom  these  experiments  it  is  assumed  in  this 
case  that  the  blood  supplies  of  both  choroid  and  retina  were  interrupted. 
If  this  explanation  be  accepted,  why  does  blood  continue  to  circulate  in 
the  vessels?    It  is  a  clinical  fact,  which  I  have  witnessed,  that  after  a  t^mor 


AFFIXTIOXS    OF    THE    CEIOKOID.  :>  3 

of  the  optic  nerve  has  been  cxilsed,  and  all  \essels  which  enter  the  eyi 
posteriorly  cut,  a  meager  circulation  reappears  in  the  retina  within  a  few 
hours.  In  such  cases  it  is  assumed  that  the  blood  enters  the  choroid 
through  the  recurrent  branches  of  the  anterior  ciliary  arteries  and  reaches 
the  retina  through  the  coUaterils  around  the  head  of  the  nerve  (circle  oi 
Zinn).* 

DiA(;N()SIS.  In  this  fundus  sclerosis  of  the  choroid  is  so  prominent 
that  the  condition  of  the  nervj  and  retina  might  easily  be  overlooked.  That 
the  origin  of  the  degeneraticns  is  traumatic  and  involves  the  optic  nerve 
is  suggested  by  the  absolute  blindness  of  the  eye  and  extreme  atrophy  of 
the  disc.  The  condition  is  differentiated  from  senile  degeneration  of  the 
choroid  bv  its  wide  extent  and  limitation  to  one  eye.  It  differs  from  exu- 
dati\'e  choroiditis  bv  its  uniform  distribution  and  absence  of  deep  choroidal 
cicatrices.  Prom  diffuse  choroiditis  the  differentiation  is  more  difficult, 
but  in  this  affection  the  lesions  are  distributed  with  considerable  irregularity 
and  the  atrophy  which  follows  is  less  uniform.  The  condition  could 
scarcely  be  mistaken  for  albinism.  Moreover,  in  none  of  the  diseases  men- 
tioned is  the  eve  completely  blind. 

Sterkogram  26.  Atrophy  of  Choroid.  Acquired  Albinism. 
(Depigmentation  of  Fundus  in  a  Diabetic).  Right  eyeground  of  a  woman 
53  years  of  age.  Mother  of  eight  healthy  children.  Although  well 
nourished  and  apparently  healthy,  the  urine  contains  9  per  cent,  of  sugar. 
Always  had  good  vision  until  two  years  ago,  when  it  began  to  fail.  Right 
eye  counts  fingers  at  three  feet.  Left  eye,  vision  is  20/70.  Visual  fields 
contain  large  irregular  defects.  There  is  neither  photophobia  nor  torpor 
retinae.  Has  never  experienced  any  subjective  symptoms  of  choroiditis, 
/'.  e.,  photopsicE,  micropsias,  etc.  Similar  fundus  changes  exist  in  both  eyes, 
but  are  furthest  advanced  in  the  right.  In  this  eye  there  is  almost  com- 
plete depigmentation  of  the  entire  fundus.  The  choroidal  vessels  appear 
with  great  distinctness  against  the  uncovered  sclera,  producing  an  oph- 
thalmoscopic picture  closely  resembling  that  of  albinism  (Stergm.  15). 
1  he  smaller  vessels  of  the  choroid  and  choriocapillaris  have  disappeared 
from  large  areas  of  the  fundus  which  now  present  a  white  reflex.  Evi- 
dently, these  choroidal  vessels  have  been  obliterated  by  a  simple  atrophic 
process,  inasmuch  as  no  evidence  of  inflammatory  action  exists  in  the  eye. 


*  Tn  tumors  of  the  optic  nerve  adventitious  vessels  form  before  operation,  vi^hich 
replace  the  central  artery  and  supply  the  retina. 


56  THE    FUNDUS    OCULI. 

The  retinal  vessels  appear  normal.*  The  disc  shows  no  sign  of  disease, 
although  it  appears  reddened  and  ill  defined  as  in  all  cases  where  it  is 
no  longer  contrasted  with  the  normal  choroid.  The  choroidal  atrophy  is 
most  marked  in  the  posterior  polar  zone  and,  in  a  general  way,  diminishes 
toward  the  periphery.  1  he  disc  is  surrounded  by  a  wide,  irregular-shaped 
ring  of  highly  atrophic  choroid.  The  choroidal  and  retinal  pigment  have 
vanished  without  migrating  into  the  retina.  On  the  temporal  side  of  the 
picture  are  three  small  groups  of  pigment  specks,  situated  among  the 
vessels  of  the  choroid.  The  macular  region  contains  a  few  reddish  spots 
which  have  partially  resisted  the  degenerative  process.  One  of  these 
contains  the  fovea  which  appears  as  a  small,  round,  red  spot.  That  this 
is  the  patient's  point  of  fixation  is  proveci  by  directing  her  to  look  at  the 
center  of  the  ophthalmoscopic  mirror,  when  this  red  spot  will  be  found 
in  the  observer's  direct  line  of  vision. 

The  iris  of  the  congenital  albino  contaiiis  no  pigment,  consequently, 
an  excess  of  light  enters  the  eye,  causing  Intense  photophobia.  In  this 
patient  the  I  rides  were  normally  pigmented  and  she  had  no  intolerance 
of  light.  These  facts  tend  to  confirm  the  theory  that  it  is  the  pigment  in 
the  iris,  not  that  in  the  fundus,  which  prevents  dazzling  in  the  normal  eye. 

The  pathologic  changes  in  this  eye  may  be  attributed  to  the  existing 
diabetes,  a  disease  known  to  cause  degeneration  of  the  ocular  vessels. 
When  diabetes  affects  the  vessels  of  the  retina,  the  fundus  presents  the 
picture  of  retinitis.  It  may  be  asked,  why  degeneration  of  the  retinal 
vessels  should  be  attended  by  edema,  exudation  and  hemorrhage,  while 
degeneration  of  the  choroidal  vessels  produces  none  of  these  phenomena. 
The  explanation  is  found  in  the  following  anatomic  variations.  The 
retinal  vessels  have  no  anastomoses;  hence,  obstructive  disease  is  followed 
by  sudden  disaster.  The  choroid,  on  the  contrary,  is  a  vascular  plexus 
whose  free  anastomoses  are  a  protection  against  loca^  ischemia,  edema  or 
hemorrhage  in  case  a  vessel  is  obstructed.  Although  the  vessels  of  this 
choroid  are  being  obliterated  by  angiosclerosis.  their  walls  remain  invisible, 
differing  in  this  respect  from  the  familiar  forms  of  choroidal  sclerosis  in 
which  the  walls  of  the  vessels  become  very  white.  Both  Raehlmann  and 
Coats  regard  translucent  thickening  of  a  vessel's  walls  as  due  to  disease 
of  the  inner  coat  ( intima ) ,  and  opaque  thickening  of  the  walls  to  changes  in 


*  It  was  observed  as  a  curious  physical  phenomenon,  that  the  arteries  exhibited 
central  light  streaks  only  where  they  crossed  the  nerve  or  in  regions  of  the  fundus 
vvhicli  furnished  a  reddish  background. 


MM. CTIOXS    OF    Tin:    CHOROID.  57 

the  external  coat  (adventitia) .  Opaque  vessels  are  also  rare  in  diabetic 
retinitis  although  profound  vascular  disease  is  present  as  shown  by  the 
number  of  hemorrhages  (Stergm.  38).  The  depigmentation  of  this  fundus 
may  be  analogous  to  the  discharge  of  pigment  from  the  iris  and  ciliary 
body  that  follows  iridectomy  in  a  diabetic  subject.  In  such  cases  the 
amount  of  pigment  discharged  may  cause  the  aqueous  to  look  like  ink. 

Diagnosis.  This  rare  fundus  condition  is  presented  for  comjrarison 
with  other  forms  of  choroidal  atrophy,  from  which  it  is  differentiated 
chiefly  by  translucent  atrophy  of  the  vessels  and  destruction  of  pigment. 
It  may  be  distinguished  from  congenital  albinism  by  recent  failure  of  vision, 
normal  pigmentation  of  other  parts  and  atrophy  of  the  choroidal  vessels. 
The  eye  of  the  albino  is  normal  except  that  it  contains  no  pigment. 

Stereogram  27.  Senile  Changes  in  thi:  Fundus.  Colloid  Ex- 
crescences (Drusen).  Macular  Degeneration.  Arteriosclerosis. 
Left  eyeground  of  a  woman  73  vears  of  age.  Similar  fundus  cond'tinns 
present  in  both  eyes.  Vision  20/50.  The  region  about  the  nerxe  a:ul 
macula  is  covered  with  superficially  situated,  round,  yellowish  dots,  none  of 
which  exceeds  in  si/e  the  width  of  the  central  vein.  They  are  confined  to 
the  posterior  polar  zone  and  occur  chiefly  on  the  temporal  side  of  the 
nerve.  The  dots  occur  singly  anci  in  groups.  Some  of  them  are  closely 
crowded  together,  but  show  no  tendency  to  coalesce  and  form  large  masses. 
They  are  always  below  the  retinal  vessels,  but  some  of  the  larger  dots 
appear  elevated  above  the  surface  of  the  retina.  Neither  the  dots  nor 
surrounding  fundus  are  pigmented.  At  the  macula  the  foveal  pit  is  sur- 
rounded bv  a  ring  of  fine,  granular  pigment.  The  optic  nerve  contains  a 
deep  physiologic  excavation  with  a  very  white  floor,  on  which  markin<Ts 
of  the  lamina  cribrosa  are  very  distinct.  These  apnearances  are  A^'ithin 
phvsic^ogic  limits  and  the  nerve  may  be  considered  normal.  Art^rio- 
scltfro-^is  of  the  retinal  N'essels  is  indicated  bv  undue  brightness  of  the 
central  light  streak  on  all  arteries  and,  particularly,  bv  the  white  walls  seen 
on  an  ascending  artery  as  it  crosses  a  vein.  All  the  morbid  changes  ob- 
served in  this  eye  are  degenerative  in  character  and  incident  to  old  age. 

The  yellow  dots,  so  conspicuous  in  this  fundus,  are  known  under  the 
various  names  of  colloid,  hyaline,  and  wartv  excrescences,  guttate  cho- 
roiditis, colloid  degeneration  of  the  choroid,  etc.  Recent  English  writers 
have  adopted  the  German  name  "  drusen  "  to  designate  these  bodies. 
A  few  colloid  bodies  are  ophthalmoscopicallv  visible  in  the  eyes  of  most 
very  old  people.  Occasionally  they  appear  in  great  numbers,  principally 
at  the  posterior  pole.     Similar  bodies  are  seen  in  all  forms  of  pigmentary 


58  THE    FUNDUS    OCULI. 

degeneration  of  the  choroid  and  they  are  supposed  to  constitute  the  spots 
found  In  retinitis  punctata  albescens.  These  dots  do  not  themselves  Injure 
vision,  but  frequently  are  associated  with  senile  degeneration  of  the  retina 
or  choroid  In  which  case  vision  may  be  very  low. 

In  the  eyes  of  people  over  50  years  of  age  and  In  eyes  which  have 
undergone  degenerative  changes,  the  microscope  nearly  always  reveals  the 
presence  of  small,  globular  or  hemispheric  elevations,  situated  on  the 
inner  surface*  of  the  lamina  vltrea  which,  from  their  appearance,  have 
been  known  as  colloid  or  hyalln  bodies  (Figs.  56-57).  On  their  inner 
surface,  these  bodies  are  completely  or  partly  covered  by  the  pigmented 
retinal  epithelium  to  which  they  are  Intimately  connected.  They  may 
appear  to  form  a  part  of  the  lamina  or  be  connected  only  by  a  narrow 
neck.  Sometimes  they  are  found  free  In  the  retina.  Loosely  attached 
and  free  drusen  are  supposed  to  have  separated  themselves  from  the 
lamina,  but  there  Is  no  evidence  that  such  bodies  were  ever  connected  with 
it.  Colloid  bodies  may  lose  their  covering  of  pigmented  epithelium  in 
which  case  they  become  ophthalmoscopically  visible.  On  the  other  hand, 
an  eye  mav  contain  a  great  number  of  colloids,  but  if  covered  with  pigment 
they  cannot  be  seen  with  the  ophthalmoscope. 

The  origin  and  nature  of  these  formations  have  not  been  definitely 
determined.  In  structure  they  resemble  the  lamina  vltrea  and,  by  some, 
are  regarded  as  a  proliferative  thickening  of  that  membrane.  Others 
consider  them  as  degenerated  epithelial  cells.  The  lamina  vltrea  is  a 
homogeneous  membrane  deposited  by  the  pigmented  epithelium  and  it  Is 
improbable  that  it  possesses  proliferative  power.  It  is  more  reasonable 
to  suppose  that  the  excrescences  on  Its  surface  originated  from  the  same 
source  as  the  membrane,  /.  e.,  that  they  are  a  deposit  from  the  cells  of  the 
pigmented  epithelium.  In  diseased  eyes  the  colloids  are  deposited  by  the 
cells  In  response  to  an  Irritant. 

The  pigment  which  surrounds  the  fovea  In  this  eye  marks  the  early 
stage  of  senile,  chorioretinal  atrophy.  The  macula  of  the  aged  frequently 
exhibits  pigment  and  irregular,  yellowish  spots.  These  changes  depend 
on  vascular  degeneration  of  the  chorlocaplllaris,  whereby  the  retina  Is 
deprived  of  nutriment.  The  resulting  degeneration  Is  visible  chiefly  at 
the  macula,  for  the  reason  that  In  this  region  the  retina  consists  only  of 
outer  layers.     Senile  macular  degeneration  may  result  In  complete  loss  of 


*  Excrescences  never  form  on  the  outer  surface  of  the  lamina,  a  fact  which  suggests 
their  origin  from  the  retinal  epithelium. 


Fig.  55. 


Fig.  55 — Complete  Xon-glaucomatous 
Excavation  of  Disc.  Due  to  Extreme 
.Atrophy  of  the  Optic   Xerve.     Specimen 

from   an   Atro])hic    Bulb. 


Fig.  56-57 — Colloid  Degeneration 
of  tlie  Choroid.     (Drusen.) 


Fig.  57. 


AFFKCTIONS    OF    THE    CHOROID.  59 

vision.  When' senile  atrophy  of  the  choroid  extends  to  the  large  vessels, 
white,  sclerotic  patches  develop,  especially  around  the  disc  and  macula. 
The  deterioration  of  vision  in  the  case  presented  ahove  was  due  to  the 
macular  changes,  not  to  the  colloid  excrescences. 

Retinal  degeneration  is  a  constant  feature  of  old  age.  The  mem- 
brana  limitans  and  the  fibers  of  Miiller  are  greatly  thickened  and  the 
nervous  elements  may  degenerate.  In  consequence  of  these  changes  the 
retina  becomes  less  transparent  and  gives  a  stronger  ophthalmoscopic  reflex. 
The  most  constant  senile  retinal  degeneration  occurs  in  the  periphery. 
Here  the  retina  is  thickened  and  divided  by  Miiller's  fibers  into  cyst-like 
spaces  which  contain  no  trace  of  nervous  elements  (Fig.  58).  From  its 
situation  aid  from  the  appearance  of  the  spaces  this  form  has  been  termed 
peripheric  cystic  degeneration  of  the  retina,  or  Iwanoff's  edema. 

Arteriosclerosis  of  the  retinal  A-essels  is  nearly  constant  in  ad\-anced 
age.  The  changes  are  not  always  ophthalmoscopically  visible.  Senile 
sclerosis  of  the  retinal  vessels  has  not  the  same  evil  prognostic  import  as 
arteriosclerosis  from  causes  other  than  age. 

Senile  alterations  in  the  optic  nerve  are  secondary  to  either  retinal 
degeneration  or  to  arteriosclerosis. 

Diagnosis.  A  punctate  condition  of  the  fundus,  limited  to  the  pos- 
terior pole,  occurring  in  an  aged  person  is,  presumably,  due  to  colloid  ex- 
crescences. Other  punctate  conditions  which  might  be  mistaken  for  senile 
colloids,  differ  from  them  as  follows.  Gunn's  dots  (Stergm.  16)  occur  in 
the  voung,  are  smaller  in  size  and  far  less  distinct  than  colloid  bodies. 
Retinitis  punctata  albescens  (Stergm.  54)  commences  in  early  life,  is  slowly 
progressive  and  associated  with  night  blindness.  The  dots  appear  first 
at  the  periphery  of  the  fundus.  The  lesions  of  central  choroiditis  (Stergm. 
19)  are  differentiated  by  their  deep  situation,  large  size,  irregular  shape, 
presence  of  pigment  and  tenciency  to  coalesce.  Furthermore,  some  of  the 
spots  are,  obviously  exudative  in  character. 

Stereogram  28.  Rupture  of  the  Choroid.  Left  eyeground  of 
boy  10  years  of  age.  One  year  ago  the  eye  was  struck  by  a  missile  from  a 
sling.  At  that  time  the  fundus  was  obscured  by  blood  in  the  vitreous. 
Three  months  later  sufficient  blood  had  been  absorbed  to  disclose  a  large 
white  patch  around  the  nerve  but  details  were  not  discernible.  The 
stereogram  shows  the  condition  of  the  fundus  twelve  months  after  the 
injury.  The  blood  has  been  completely  absorbed  from  the  vntreous,  leaving 
the  media  clear.  An  unusually  extensive  rupture  of  the  choroid  is  seen 
completely    encircling   the    nerve.      Probably,    the    laceration    commenced 


60  THE    FUXDl'S    OCULI. 

between  the  disc  and  macula,  but  was  prolo.iged  far  beyond  the  usual  length. 
Observe  the  tendency  for  the  rupture  to  approach  the  nerve.  The  course 
of  the  rupture  is  concentric  with  the  outline  of  the  disc.  In  shape  it  is 
wide  in  the  center  and  narrower  at  the  ends.  In  the  supranasal  region 
is  a  smaller  rent  in  the  choroid  with  a  lateral  extension  and  forked  ex- 
tremity. I  he  retina  is  not  torn,  as  shown  by  its  xessels  which  pass  unin- 
terruptedly across  the  ruptures.  For  some  distance  beyond  the  rupture, 
the  retina  is  darkened  by  fine,  dust-l.ke  pigment,  signifying  that  the  pig- 
mented epithelium  was  disturbed  beyond  the  border  of  the  fracture. 
.\long  the  edge  and  over  the  surface  of  the  exposed  sclera  are  several 
groups  of  pigment.  The  pigment  along  the  edge  is  proliferating  retinal 
epithelium  which  is  endeavoring  to  cover  the  hiatus.  This  pigment  will 
progressively  increase  in  amount. 

Rupture  of  the  choroid  usually  is  produced  indireztly  by  sudden, 
violent  compression  of  the  eyeball.  In  mo:t  cases  the  force  is  applied  to 
the  front  of  the  eve  by  a  blunt  body,  like  a  fist,  a  missile,  cork  from  a  gas 
charged  bottle,  a  baU,  etc.  Exceptionally,  it  is  caused  by  force  which 
fractures  the  skull.  The  location  and  form  of  the  rupture  are  character- 
istic. Almost  invariably  it  occurs  at  the  posterior  pole,  within  two  or 
three  disc  diameters  of  the  nerve  (Colored  Plate  3).  Statistics  indicate 
that  It  is  about  five  times  i^.ore  frequent  on  the  temporal  than  on  the 
nasal  side.  Ruptures  confit^d  to  the  re3;io:i  above  or  below  the  nerve  ar2 
uncommon.  The  favorite  site  is  between  the  macula  and  disc.  Immediatelv 
following  the  accident  there  is  hemorrhage  which  usually  enters  the 
vitreous  and  h'des  the  fundus.  Vision,  of  course,  is  interrupted.  After 
resorption  of  the  blocd,  rupture  of  the  choroid  is  recognized  by  discovery 
of  a  white,  crcsc2::tic  fi-^ure  at  the  posterior  pole,  the  long  diameter  of 
which  is  concentric  with  the  margin  of  the  disc.  The  borders  of  the 
rupture  are  generally  pigmented.  Blood  may  remain  in  the  rent  for  a  lon^^ 
time.  The  rupture  may  be  either  single  or  multiple.  Frequently  the  rent  is 
forked  or  branched.  The  tear  involves  the  entire  thickness  of  the  choroid 
and  laver  of  pigmented  epithelium.  The  suprachoroidea  also  is  torn,  but 
shreds  of  this  membrane  may  remain  in  the  wound,  attached  to  the  sclera. 
Yellowish  streaks,  in  fcrm  n:-"^  '^osition  resembling  ruptures,  may  follow 
trauma  or  accompany  -lacerations  of  the  choroid.  These  streaks  are 
sunnosed  to  be  incomplete  fr'^c^'ures  of  the  choroid  and  pigmented  epi- 
thelium, which  have  not  extended  through  to  the  sclera.  Rupture  of  the 
choroid  frequently  is  comnlicted  with  dislocation  of  the  lens,  detachment 
of  the  retina,  rupture  of  the  sphincter  pupillae,  etc.     The  group  of  cases 


Fig.  58. 


Fi.^.  .59 — Rupture  of  Choroid,  Extend- 
ing into  Nerve  Sheath.  N,  optic  nerve: 
D.  detached  retina;  H,  hernia  of  retina 
into  ruptured  .sheath. 


Fig.  58 — Senile  Degeneration  of  the 
Retina. 

Cyst-like  spaces  devoid  of  nervous  ele- 
ments and  separated  by  Miiller's  fibers 
ave  formed  in  the  thickened  retina. 


Fig.  59. 


Fig.    60 — Rupture    of    Choroid    extend- 
ing into  Optic  Nerve. 


I'ig.  tJO. 


AFFIX'TIONS    OF    THEC    CHOROID.  61 

described  In  this  chapter  includes  only  those  ruptures  of  the  choroid  in 
which  injury  is  repaired  without  loss  of  the  eye.  In  a  larger  number  of 
nonperforating  injuries,  laceration  of  the  choroid  is  so  extensive  and  the 
resulting  hemorrhage  so  profuse  that  the  eye  is  irrevocably  lost  and  enu- 
cleation follows,  without  the  choroidal  lesion  ever  coming  under  observa- 
tion. In  a  number  of  such  cases,  microscopically  examined,  I  have  noted  a 
tendency  for  extensive  laceration  of  the  choroid  to  extend  to  the  edge 
of  the  nerve  or  even  into  its  substance.  A  curious  example  is  shov/n  in 
Fig.  59.  The  photomicrograph  is  from  an  eye  which,  thirteen  months 
before  enucleation,  was  blinded  by  a  blow  from  a  stone  projected  by  a 
blast.  Microscopic  examination  shows  an  extensive  fracture  of  the  cho- 
roid which  is  prolonged  through  the  sclerochoroidal  ring  into  the  inter- 
vaginal  space  around  the  nerve.  Through  the  aperture  thus  created  a 
knuckle  of  detached  retina  has  prolapsed  into  the  sheath  of  the  nerve, 
forming  a  hernia.  Figure  60  is  from  an  eye  enucleated  ten  days  after 
violent  application  of  blunt  force  to  the  ciliary  region.  Xo  external  rupture 
of  the  globe.  Microscopic  examination  revealed  a  detached  retina,  large 
subretlnal  blood  clot  and  a  broad  rupture  of  the  choroid,  one  end  of  which 
extended  into,  and  partly  through,  the  head  of  the  o[)tic  nerve. 

It  Is  difficult  to  understand  whv  rupture  of  the  choroid  always  occurs 
at  the  posterior  pole,  usuallv  on  the  temporal  side,  and  follows  a  cir- 
cumpapillary  course.  The  number  of  theories  offered  In  explanation  is 
a  measure  of  their  inadequacy.  These  are,  (1)  coiilre  coup;  (2)  driving 
the  eyeball  over  the  ner\'e;  (3)  rotation  of  globe  which  suddenly  is 
arrested  by  the  optic  ner\e  or,  (4)  bv  the  short  ciliary  arteries.  The 
position  of  the  classic  rupture  of  the  choroid  corresponds  to  the  posterior 
border  of  the  oblique  muscles  and  it  is  probable  that,  when  the  sclera  and 
attached  choroid  are  forcibly  driven  backward,  these  muscles  contract 
spasmodically,  and  that  the  choroid  is  broken  acrt)ss  their  rigid  edge. 
The  retina  escapes  laceration  because  it  is  free  and  unattached. 


Chapter  V. 

SCLEROSIS  OF  THE  RETINAL  VESSELS  (JNGIOSCLEROSIS) 
AND  RETINAL  HEMORRHAGE. 

In  the  following  paper  an  eliort  has  been  made  to  present  the 
pathologic  events  of  arteriosclerosis  in  sequence,  although,  clinically,  no 
disease  is  more  erratic  in  its  course. 

The  first  ophthalmoscopic  evidences  of  sclerosis  of  the  retinal 
vessels  are  dilation,  tortuosity  anci  pulsation  of  the  arteries.  Thoma  con- 
siders the  dilation  as  due  to  loss  of  elasticity  and  contractility  in  the 
walls.  Naturally,  dilation  is  followed  by  tortuosity.  The  pulsation  is 
characterized  by  a  lateral  displacement  of  the  whole  artery,  most  marked 
at  bends  and  curves.  When  present,  it  is  well  developed  and  unmistak- 
able. It  is  a  true  arterial  pulsation  termed  locomotion  pulse  as  distinguished 
from  the  pressure  pulse  seen  in  glaucoma  and  whenever  general  blood 
pressure  sinks  below  intraocular  pressure.  Locomotion  pulse  occurs  in  all 
cases  of  incipient  retinal  angiosclerosis  in  which  the  arteries  are  pri- 
marily affected,  but  as  it  causes  no  disturbance  of  vision  it  is  seldom  seen 
by  the  ophthalmologist.*  As  vascular  degeneration  progresses  and  the 
arteries  become  rigid,  arterial  pulsation  disappears.  Observations  are 
lacking  as  to  the  transparency  of  the  arterial  walls  in  the  first  stage  of  ar- 
teriosclerosis, but  in  the  cases  which  I  have  recognized,  the  walls  of  the 
arteries  were  distinguishable  by  weak  illumination. 

Stereogram  29.  Diffuse  Retinal  Arteriosclerosis  Primary 
Stage.  Bov,  15  vears  of  age.  Sought  advice  because  of  recurring  epis- 
taxis.  Incidentally,  the  eyes  were  examined  when  a  probable  cause  for 
the  bleeding  was  discovered  in  the  condition  of  the  blood  vessels.  The 
retinal  nrteries  were  slightly  dilated  and  more  tortuous  than  the  veins. 
The  smiller  Avascular  twigs  were  very  distinct.  The  arterial  blood  col- 
umns had  extremely  delicate,  light  borders,  evidently,  a  slight  opacity  of 
the  Avails.  The  most  conspicuous  feature  in  the  fundus  was  a  strong  arterial 
pulse,  especially  pronounced  wherever  an  artery  made  a  sharp  bend.  The 
veins  were  of  normal  size  and  not  unduly  tortuous.     The  optic  disc  was 


Suggests  the  necessity  of  using  the  ophthalmoscope  in  general  diagnosis. 

62 


SCLEROSIS    or    REIINAL    VESSELS HEMORRHAGE.  6^ 

slightly  reddened.  Urine  was  found  to  contain  albumin  and  hyalin  casts. 
Heart  slightly  dilated  but  no  murmurs.     Blood  pressure  was  120  mm.  Hg. 

In  the  early  stage  oi  typical,  retinal  arteriosclerosis  the  veins  are 
unchanged  and  as  a  rule  are  less  tortuous  and  no  broader  than  the  arteries. 
This  disease,  ho\ve\'er,  is  capricious  and  the  veins  may  be  attacked  before 
the  arteries,  in  which  event  there  is  venous  turgescence  and  no  pulsation 
in  the  arteries.  In  the  case  described  above,  the  blood  pressure  was  not 
elevated.  Probably,  it  always  is  normal  or  subnormal  in  the  stage  of 
arterial  relaxation  and  dilation.  Later  in  the  disease  when  the  arteries 
grow  rigid  and  the  terminals  unyielding,  blood  pressure  will  be  increased. 
In  very  early  arteriosclerosis,  the  relaxed  blood  vessels  absorb  much  of 
the  force  imparted  to  the  pulse  wave. 

Degeneratixe  changes  in  the  arteries  always  are  accompanied  bv 
analogous  changes  in  the  veins,  although  the  disease  may  be  further  ad- 
vanced in  one  than  in  the  other.  Nearly  all  the  retinal  vessels  may  exhibit 
disease.  More  often,  however,  the  pathologic  changes  are  limited  to  a 
certain  vascular  circuit  or  even  to  a  single  vessel.  Angiosclerosis  is  as- 
sociated with  a  variety  of  chronic  diseases,  to  some  of  which  it  bears  a 
causative,  and  to  others  a  secondary  relation.  In  any  event,  the  disco\-ery 
of  vascular  degeneration  in  the  retina  is  an  omen  of  gravest  significance. 

Opportunities  for  the  microscopic  study  of  early  arteriosclerosis  are 
rare,  consequently  little  is  known  of  the  histologic  changes  occurring  in 
the  stage  of  arterial  relaxation  and  dilation.  Mott  considers  degenera- 
tion of  the  elastic  and  muscle  fibers  as  the  primary  lesion.  Jores  says 
that  arteriosclerosis  commences  by  formation  of  spaces  in  the  elastica,  at 
the  period  of  time  corresponding  to  arterial  dilation.  Ribbert  also  de- 
scribes the  first  change  as  spacing  of  the  intima.  Thoma  saw  the  starting 
point  in  a  noninflammatory  swelling  of  the  media.  Vasomotor  paralysis  has 
been  advanced  as  a  cause  of  the  early  relaxation.  This  appears  improbable; 
at  least,  section  of  the  cervical  sympathetic  in  animals  does  not  affect  the 
retinal  vessels. 

Etiology.  No  age  is  exempt  from  arteriosclerosis,  but  In  infancy  it 
is  rare,  while  as  a  manifestation  of  senility  it  is  constant.  It  is  much  more 
prevalent  among  young  adults  of  both  sexes  than  is  generally  supposed. 
It  is  common  among  muscular,  hard-working  individuals  and  comparatively 
rare  among  the  feeble.  Vascular  disease  is  more  common  among  men 
than  women,  but  not  where  women  perform  the  same  manual  labor  as 
men  and  not  after  the  climacteric.  It  is  very  frequent  among  those  who 
labor  in  a  cold,  damp  atmosphere.    The  most  important  factor,  how-ever,  is 


64  THE    FUNDUS    OCULI. 

heredity,  manifested  in  certain  families  by  a  tendency  to  vascular  degenera- 
tion from  slight  causes  and  by  premature  senility.  Arteriosclerosis  is  inti- 
mately associated  with  chronic  nephritis  and  slight,  vascular  changes  in  the 
retina  may  antedate  the  appearance  of  albumin  in  the  urine.  Prominent 
causative  diseases  are  gout,  articular  rheumatism  and  syphilis.  Vascular 
degeneration  often  is  traceable  to  the  toxic  action  of  lead,  alcohol,  tobacco, 
phosphorous  and  other  drugs.  Many  recent  writers  regard  intestinal 
autointoxication  as  one  of  the  common  causes  of  this  disease.  Infectious 
vasculitis  may  occur  in  any  infection. 

Diagnosis.  Opportunities  seldom  occur  for  diagnosticating  arterio- 
sclerosis during  the  stage  of  arterial  relaxation.  Simple  association  of 
tortuous  arteries  with  a  locomotion  pulse  is  almost  pathognomonic  of 
arteriosclerosis.  Aortic  regurgitation,  aneurism  and  Basedow's  disease  may 
also  show  locomotion  pulsation  and  these  diseases  must  be  excluded  in 
making  the  diagnosis.  As  distinguished  from  the  locomotion  pulse,  which 
occurs  in  the  arteries  throughout  the  retina,  a  pressure  arterial  pulse  is  con- 
fined to  the  disc.  Pulsation  in  the  retmal  arteries  should  not  be  diag- 
nosticated unless  it  is  distinct  and  unmistakable.  Slight  movements  of 
the  patient's  eye  or  of  the  ophthalmoscope  are  easily  mistaken  for  pulsation 
in  the  \essels. 

As  first  described  by  Thoma,  thinning  and  distension  of  the  vessel 
walls  in  arteriosclerosis  is  soon  arrested  by  proliferat'on  and  thickening 
of  the  intima,  media  and  adventitia,  a  process  regarded  as  compensatory. 
Witli  the  advent  of  this  process  the  disease  enters  the  stages  of  vascular 
rigidity  and  degeneration,  the  pathology  of  which  includes  nearly  all  morbid 
changes  that  occur  in  the  inner  layers  of  the  retina.  The  chief  oph- 
thalmoscopic features  of  the  degenerative  stage  are  changes  in  the  color 
of  the  vessels,  opacities  in  their  walls  and  abnormal  variations  in  the  size 
of  t'^e  blood  columns.  The  arteries  as  a  whole  are  somewhat  lighter  in 
ccijr  than  normal.  In  addition,  the  central  light  streak  becomes  brighter, 
broader  and  extends  unusually  far  out  into  the  periphery.  When  this  ap- 
per.rr.nce  is  pronounced,  the  vessels  have  been  aptly  compared  to  bur- 
nished wire  and  are  called  "silver  wire  arteries"  ( Stergm.  34).  The 
sclerosed  v/all  of  a  retinal  vessel  may  or  may  not  become  opaque.  A 
no'—'al  retinal  vessel  has  transparent  walls.  It  is  the  column  of  blood 
w'th'n  t''!e  \'essel  and  not  the  vessel  itself  that  is  seen.  Therefore,  an 
opacity,  however  slight,  connected  with  a  blood  column  should  be  re- 
garded as  abnormal.  When  the  walls  of  a  vessel  become  visible  the 
blood  col nmn  aopears  bordered  by  light  lines,  the  distinctness  of  which 


SCLEROSIS    OF    RETINAL    VESSELS HEMORRHAGE.  65 

is  governed  by  the  degree  of  opacity.  Of  course,  the  opacity  is  not 
limited  to  the  lateral  walls,  but  is  visible  only  there,  because  the  light  from 
the  blood  column  renders  translucent  a  moderate  opacity  in  the  overlying 
wall.  Also,  on  the  sides  of  the  vessel  we  are  looking  through  a  much 
greater  thickness  of  tissue  than  on  the  top.  The  degree  of  opacity  in 
the  walls  of  a  retinal  vessel  is  no  measure  of  the  thickening  which  exists. 
In  fact,  the  walls  may  be  thickened  even  to  the  ix)int  of  oblitering  the 
lumen  without  becoming  visible.  Thickening  and  opacity  of  the  walls  is 
first  distinguishable  and  most  pronounced  where  an  artery  crosses  over 
a  vein.  Under  normal  conditions  the  walls  of  an  artery  do  not  hide  an 
underlying  vein,  and  the  vein  can  be  traced  up  to  the  blood  column  of  the 
artery  and  sometimes  may  be  faintly  seen  as  it  passes  beneath.  If,  how- 
ever, the  walls  of  the  overlying  artery  are  opague  they  obscure  the  vein 
and  the  course  of  the  latter  appears  to  be  interrupted  at  a  short  distance 
on  either  side  of  the  artery  (Stergms.  30-31-32).  Frequently,  an  under- 
lying vein  is  hidden  by  an  artery  with  thickened  but  translucent  walls. 
In  such  cases  an  open  space  appears  to  separate  the  ends  of  the  vein, 
which  seem  to  be  pointed,  from  the  artery.  Sometimes  a  sclerosed  artery  is 
visible  beneath  a  thin  normal  vein.  As  a  rule,  opacities  extend  only  for 
short  distances  along  a  vessel.  Opacities  often  appear  as  plaques  or  brig^it 
s]x)ts  in  the  walls. 

Arteriosclerosis  shows  a  preference  for  the  large  vessels  on  the 
disc,  especially  the  central  artery  and  vein  at  their  point  of  bifurcation 
(Fig.  61).  Arteriosclerotic  changes  on  the  disc,  however,  should  be 
diagnosticated  with  caution,  inasmuch  as  it  is  a  common  congenital  con- 
dition for  the  large  vessels  on  the  disc  to  be  ensheathed  with  connective 
tissue,  which  a])pears  as  narrow  white  lines  bordering  the  arteries  and 
veins.  As  a  rule,  this  tissue  only  extends  to  or  slightly  beyond  the  edge  of 
the  disc.  As  wall  degeneration  advances  the  blood  column  becomes  ir- 
regularly narrowed.  Translucent  thickening  of  the  wall,  with  greatly 
narrowed  blood  column,  limited  to  short  sections,  is  said  to  indicate 
nodular,  endothelial  proliferation  (endarteritis  nodosa). 

Stereogram  30.  Advanced  Sclerosis  oe  Retinal  Vessels. 
Left  fundus  oculi  of  a  laboring  man,  59  years  of  age,  with  ad- 
vanced general  arteriosclerosis.  Urine  normal.  Marked  arteriosclerotic 
changes  appear  in  both  retinae,  especially  the  left.  The  disc  has  a  brick- 
red  color.  The  arteries  are  narrowed  but  not  tortuous.  The  walls  of  the 
primarv  subdivisions  of  the  central  artery  are  thickened  but  translucent 
as    shown    where   thev   cross   over  a  vein.       At  such  points  the    underlying 


66  THE    FUNDUS    OCULI. 

vein  is  completely  obscured  for  a  short  distance  on  each  side  of  the 
artery,  but  the  walls  of  the  artery  are  not  visible  with  a  bright  illumi- 
nation. This  form  of  thickening  is  supposed  to  indicate  endothelial  pro 
liferation  in  the  artery.  In  the  inferior  temporal  vein  the  blood  column 
is  irregularly  narrowed  and,  in  places,  bordered  by  white  lines.  Thes? 
linear  opacities  are  supposed  to  be  in  the  outer  walls  and  imply  that  the 
sclerosing  process  has  extended  to  the  media  and  adventitia.  The  veins 
are  tortuous,  dilated  and  broadened  peripherally.  In  passing  below  a 
sclerosed  artery  they  appear  narrowed  and  disappear.  The  most  con- 
si:  icucus  changes  are  found  in  the  superior  temporal  vein.  Between  the 
disc  a:d  point  of  bifurcation,  the  vein  is  extremely  tortuous  and  greatly 
narrowed  except  at  certain  places  which  are  ectatic.  Beyond  the  point 
of  bifurcation  the  branches  of  the  vein  are  abruptly  and  enormously  di- 
lated. A  great  number  of  small  collateral  veins  have  developed  along  the 
constricteci  portion.  Throughout  the  area  to  which  this  vein  is  distrib- 
uted, the  retina  is  opaque  anci  contains  old  and  recent  hemorrhages. 
The  meshwork  {iviindcrnetzc)  of  little  veins  consists  of  dilated  venules 
which  ^have  established  a  collateral  flow. 

Diagnosis.  The  ophthalmoscopic  diagnosis  of  retinal  arteriosclerosis 
is  made  only  after  a  successful  search  for  some  of  the  vascular  changes 
described  above.  A  common  error  is  mistaking  a  congenital  connective 
tissue  sheath,  on  a  disc  vessel,  for  a  pathologic  condition.  The  con- 
genital tissue  is  limited  to  the  disc  and,  although  very  dense,  the  retinal 
vessels  are  perfectly  normal.  Were  such  dense  opacity  due  to  arterio- 
sclerosis it'  would  be  accompanied  by  unmistakable  evidence  of  disease 
elsewhere  in  the  retina.  Healthy  retinal  vessels  are  very  compressible, 
while  sclerosed  vessels  are  comoaratively  incompressible.  These  facts 
may  be  utilized  in  diagnosis.  Thus,  if  gradually  increasing  pressure  is 
applied  by  the  finger  to  the  eye  the  following  phenomena  may  be  observe(' 
with  the  ophthalmoscope:  (1)  strong  venous  pulsation  appears  en  the 
disc;  (2)  arterial  pulsation;  (3)  cessation  of  circulation  and  blanchin;? 
of  the  vessels.  Rijiid  vessels,  on  the  other  hand,  resist  pressure  and. 
where  retinal  arteriosclerosis  is  adv^anced,  neither  Aenous  pulsation  nor 
blanching  of  the  vessels  can  be  produced  by  pressing  on  the  globe  with 
the  finger.  The  ease  with  which  the  retinal  circulation  can  be  arti- 
ficially arrested  is  a  measure  of  arterial  tension  and  mural  rigidity. 
Meh-ille  Black  states  that  where  the  heart  is  dilated  and  the  blood  pressure 
low.  pressure  on  the  globe  causes  decided  emptying  of  the  arteries  with 
but  little  change  in  the  veins.     There  are  certain  external   ocular  mani- 


Fig.  61. 
Sclerotic  Changes  in  tlie  Central  .Artery  and  Vein  at  their  Bifurcation  on  t'le  Disc. 


s?^-^^^'^>|:^^^- 


^mammi^^^^^^ 


Fig.  62. 
Distension  of  Retinal  Vein,  due  to  Pressure  from  the  Sclerosed  and  Rigid 
Artery   Overlying. 


SCLEROSIS    OF    RETINAL    XESSE'.S HEMORRHAGE.  67 

festations  of  arteriosclerosis  of  considerable  diagnostic  value.  Spon- 
taneous subconjunctival  hemorrhage,  ocular  or  palpebral,  occurring  in 
people  over  35  vears  of  age,  usually  indicates  vascular  degeneration. 
The  same  is  true  of  fugitixe  edema  of  the  lids.  Hirschberg  first  noticed 
that  persons  with  arteriosclerosis,  especially  of  the  cerebral  Nessels,  were 
subject  to  recurring  chemosis  of  the  conjunctiva.  This  was,  presumably, 
caused  by  closure  of  small  branches  from  the  anterior  ciliary  vessels. 
White  lines,  due  to  distension  of  the  peri\ascuLir  sheaths,  border  the 
\essels  in  early  neuritis  (Stergm.  65)  and  after  sc\ere  concussions  of 
the  retina  (Stergm.  59).  Such  lines  are  differentiated  from  arteriosclerotic 
opacity  by  their  situation  on  and  near  the  disc,  by  the  evid-^ntly  acute  nature 
of  the  process  and  by  the  history.  Whei  intraocular  pressure  is  increased 
by  pressing  on  the  eye  with  the  finger,  pulsation  occurs  in  the  retinil  veins 
and  arteries.  Ihe  degree  of  pressure  necessary  to  cause  pulsation  is  diag- 
nostic; thus,  in  normal  eves  it  is  produced  by  moderately  firm  pressure;  in 
glaucoma  it  appears  when  the  eye  is  barely  pressed,  but  it  caiinot  be  induced 
in  a  sclerosed  artery  by  any  degree  of  compression. 

The  effect  of  high  blood  pressure  in  the  retina  is  felt  especially  in  the 
terminal  venous  twigs,  which  become  distended,  tortuous  a  id,  apparently, 
increased  in  number.  Retinal  arteries  do  not  retain  the  tortuosity  ac- 
(]uired  in  the  early  stage  of  arteriosclerosis.  As  the  arter  al  walls  be- 
come rigid  from  prolileration  of  new  tissue,  tortuosity  decreases  in  the 
arteries  and  increases  in  the  veins.  Raehlmann  observed  that  tortuosity 
of  the  retinal  arteries  was  most  pronounced  where  general  arteriosclerosis 
was  but  slightly  de\-eloped,  and  entirely  absent  in  the  most  highly  de- 
veloped cases  of  this  disease.  Arterial  pulsation  also  disappears  as  rig- 
idity de\elops  in  the  arteries.  Raehlmann  describes  a  characteristic  type 
of  venous  pulsation  usually  present  in  advanced  arteriosclerosis.  Other 
observers,  including  the  writer,  have  been  less  fortunate  than  Raehlmann 
in  discovering  this  pulse.  In  passing  under  a  sclerosed  artery  a  vein 
dips  with  a  sharp  twist  beneath  it  and  at  the  same  time  appears  to  narrow. 
The  pointed  appearance  which  a  vein  assumes  as  it  passes  beneath  a 
semiopaque  artery  is  an  optical  e*^ect  produced  by  the  walls  of  the  over- 
lying artery  which  gradually  obscure  the  vein.  A  rigid  artery  lying  across 
a  flaccid  vein  may  interfere  with  the  venous  circulation,  causing  distension 
of  the  vein  distal  to  the  point  of  pressure  (  Fi^^.  62).  The  veins  may  be 
dilated  in  the  peripheral  parts  and  narrowed  at  the  disc.  This  is  due 
partly  to  thickening  of  the  venous  walls  at  the  nerve  and  partly  to  ac- 
cumulation of  blood  in  the  terminals  and  capillaries.     Varicosities  are  fre- 


68  THE    FUNDUS    OCULI. 

quent,  appearing  as  dark,  oblong  enlargements  or  knob-like  protuberances 
in  the  course  of  a  vein.  Aloderate  venous  distension  may  be  the  logical 
result  of  arterial  rigidity,  but  when  extreme,  it  probably  depends  upon 
obstructive  endophlebitis  in  the  central  vein  or  branches  (Fig.  63).  Mil- 
iary aneurisms  form  on  the  small  arterioles  and  capillaries.  In  the  larger 
vessels  local  dilations  occur  where  the  walls  are  least  affected.  Frequently, 
they  form  immediately  behind  a  rigid  constriction.  It  is  doubtful  if  pul- 
sating retinal  aneurisms  occur  in  the  retina  except  as  the  result  of 
traumatism. 

Morbid  An\tomy.  Within  the  retina  the  arteries  possess  a  thick 
wall  containing  elastic  fibers  but  no  elastic  membrane.  The  veins  consist 
only  of  the  endothelial  lining  upon  a  thin  connective  tissue  wall  which 
contains  no  elastic  fibers.  Thus  by  employing  a  selective. stain  for  elastic 
tissue  the  retinal  arteries  are  readily  differentiated  from  the  veins.  In 
the  retina  two  forms  of  vascular  degeneration  predominate.  In  One,  the 
intimal  cells  proliferate  and  form  nodular  or  elongated  masses  on  the 
inner  wall  ol  the  vessel.  Usually  these  deposits  occur  only  on  one  side, 
thereby  causing  eccentric  narrowing  of  the  kimen.  Exceptionally,  nodular 
growths  spring  from  opposite  sides,  reducing  the  lumen  to  a  central  slit 
(Artery  in  Fig.  64).  In  the  other  form  of  degeneration  the  walls  of 
the  vessels  thicken  from  proliferation  of  connective  tissue,  which  narrows 
the  lumen  concentrically  (Fig.  65).  The  latter  process  is  known  as  fibrosis 
and  usually  terminates  in  hyaloid  degeneration  (Vein  in  Fig.  64).  These 
two  forms  of  degeneration  often  are  combined.  An  artery  or  a  vein  may  be 
affected  with  either  type,  but  the  arteries  tend  to  endothelial  proliferation 
and  the  veins  to  fibrosis.  Sometimes  the  perivascular  sheath  of  a  sclerosed 
vessel  is  dilated  (Tig.  66).  The  lesion  of  syphilitic  arteries  is  a  dense, 
round  celled  infiltration  in  and  around  the  outer  walls  (Fig.  67)  which  is 
followed  by  formation  of  cicatricial  tissue  (Fig.  68).  Recently,  it  has 
been  observed  that  organs  and  tissues  to  which  arteriosclerotic  vessels  are 
distributed  undergo  fibrosis  and  hyaloid  degeneration  coincident  with  de- 
generative changes  in  the  arteries.  It  is  uncertain  whether  these  tissue 
changes  are  wholly  secondary  to  the  vascular  disease  or  whether  both  vessel 
and  tissue  changes  result  from  the  action  of  the  same  morbid  influence. 
The  deleterious  effects  of  arteriosclerosis  in  the  retina  appear  to  result 
from  impaired  nutrition  due  to  obstructions  in  the  vessels,  and  its  functional 
activity  does  not  appear  to  suffer  so  long  as  circulation  is  not  interrupted. 
It  is  possible,  however,  that  the  retinitis  of  albuminuria  and  diabetes  may, 
in  part,   depend  upon  the  local  action  of  a  toxin.      Coats   suggests  that 


Fig.  63 — Oh-triut:\  .■  F.n  1    -.ilileb:!-,,. 


Fig.  6.^. 


Fig.  64 — Nearly  Complete  Ob- 
struction of  Retinal  Artery  (shown 
to  the  left  in  the  section)  clue  to 
Xoclular  Growths  Springing  from 
Opposite  sides  of  its  Wall.  The 
Wall  of  the  Vein  (shown  to  the 
right)  is  Undergoing  Hyaloid  De- 
generation as  the  Result  of  Fibro- 
sis. 


Fig.  (>4. 


Fig.    6.5 — Concentric    Narrowing   of   the    Lumen    from 
Proliferation  of  Connective  Tissue. 


Fig.  65. 


"Fi"-.  ■  6fi — Dilati'Mi     of     the     Perivascular     Sheatli     of 
clerosed  Vessel. 


Fig^.  67 — Dense,  Round  Cell  Infiltration 
of  the  Outer  Vascular  Walls,  due  t  .< 
Syphilis. 


Fig-.  67. 


Fi.?.  68 — Secondary  Cicatricial  Formation 
in  Vascular  Walls  as  the  result  of  Syphilis. 


SCLKKOSIS    OF    RKIINAL    VKSSEI.S H  IiM(  )KK  [r\(;!:.  69 

disease  of  the  intiiiia  may  be  caused  by  a  toxin  circulating  in  the  blood  while 
thickening  of  the  outer  wall  is  compensatory  to  meet  high  blood  pressure. 

I  he  new-formed  tissue  in  the  walls  of  a  vessel  may  undergo  retro- 
gressive changes  and  pass  through  the  various  phases  of  fibrous,  hyaloid, 
amyloid,  fatty  and  calcareous  degenerations.  A  wall  affected  with  fatty 
degeneration  is  easily  torn;  consequently,  hemorrhage  frequently  is  due 
to  this  cause.  In  the  hyaloid  stage  of  degeneration  both  intima  and  media 
are  replaced  by  a  glassy,  homogeneous  material  which  causes  great  re- 
duction or  even  obliteration  ot  the  lumen. 

Stkreogram  31.  Arteriosclerosis  and  Optic  Nelritis.  Right 
eyeground  of  a  man  49  years  of  age  with  general  arteriosclerosis.  Is  of 
robust  appearance  and  unusually  muscular.  Works  in  a  foundry  where  he 
is  accustomed  to  carry  heavy  weights.  lakes  several  alcoholic  drinks 
daily.  Blood  pressure,  260  mm.  Hg.  Urine  contains  a  trace  of  albumin 
but  no  casts.  Vision  20/20.  Ad\anced  sclerosis  of  the  retinal  xessels 
in  each  eye;  most  pronounced  in  the  right.  The  optic  nerve  is 
very  red,  slightly  elevated  and  its  outlines  are  obliterated.  The  retinal 
arteries  are  pale  in  color,  pursue  a  straight  course  and  are  irregularly 
narrowed.  1  he  veins  are  tortuous,  dilated  and  their  course  appears  to  be 
interrupted  wherever  they  are  crossed  by  an  artery.  The  arterial  walls 
exhibit  both  translucent  and  visible  sclerosis.  The  blood  columns  of  the 
superior  nasal  and  inferior  temporal  arteries  are  completely  hidden,  for 
short  stretches,  by  wall  opacity.  The  inferior  nasal  artery  contains  several 
slight,  fusiform  dilations  alternating  with  sharp  constrictions  in  which  the 
blood  column  is  reduced  to  a  mere  thread.  The  terminals  of  the  veins 
are  extremely  tortuous.  Varicose  dilations  (  phlebectasize)  exist  on  two 
veins,  just  below  the  disc.  The  presence  of  high  blood  pressure  and  rigidity 
of  the  vessel  walls  in  this  case  is  illustrated  by  external  pressure  on  the 
eyeball,  which  fails  to  cause  either  venous  pulsation  or  blanching  of  the 
retina,  as  would  occur  in  a  normal  eye. 

Diagnosis.  The  degenerated  retinal  vessels  in  this  fundus  furnish  the 
key  to  a  correct  diagnosis.  In  pseudoneuritis  (Stergm.  13)  and  in  con- 
genital cyanosis  (Stergm.  58)  arteries  as  well  as  veins  are  tortuous  and 
there  is  no  angiosclerosis.  Closure  of  the  central  retinal  vein  (Stergm.  46) 
is  invariably  attended  by  profuse  hemorrhage.  In  papilledema  of  cerebral 
origin  the  nerve  head  presents  a  soft,  whitish  appearance  and  the  height  of 
the  swelling  is  much  greater  than  in  this  case.  This  fundus  is  an  example  of 
angiopathic  disease,  ophthalmoscopically  indistinguishable  from  changes 
which  might  occur  in  Bright's  disease.     In  fact,  the  conditions  are  identical. 


70  THE    FUNDUS    OCULI. 

and  there  Is  no  doubt  that  vascular  degeneration  exists  in  the  kidneys 
which,  when  further  advanced,  will  cause  this  case  to  be  classed  as  one  of 
chronic  nephritis. 

Bibliography. 

•Adler.    Med.    Rec,   May   10.   1902,   p.   721 
Alleman,    American    Medicine,    Feb.    20,    1904. 
Black,    Melville    Jour.    Am.    Med.    Assn.,    July   29.    1911. 
De    Schweinitz,    Oph.    Record,    Aug.,    1906.      Trans.    Am.    Oph.    Soc,    1906.      Maryland 

Med.  J.,  1900.     Path.  Soc,  Phila.,  1906.     Intern.  Clinics,  Vol.  1,  Series  17,  1907. 
Elschnig.    Klin.    M.   f.   Augk.,    1898,   p.   2.5. 

Friedenwald,  Jour.  I\.  M.  Assn.  XVI,  1891;  A.  of  O.,  1896,  XXV,  p.  177. 
l^uchs,   A.   f.   .v.,  XI,   44(). 
Gloor,  A.  f.  A.  XXXV,  p.  ::2s. 

Gull  and   Sutton,   Med.    Clin.   Trans.,   1872,   IV,   p.   273. 
Gunn,  T.  U.   S.,  1H98. 

Haab,  WI.  Versamml.  d.  Heidgb.  Oph.  Gesellsch.,  1900. 
Heller   Munich,   med.   Wochenschr..   1889.   p.   426. 
Hertel,  Trans.  2sth   Heid.  Oph.   Soc.  p.   1.5:i. 
Hirschberg,  C.  f    p.  A.  VTTI.  p    2.     C.  f.  p.  A.  XIV,  p.  322. 

Jores,   Wesen  und    Ent'Aickelung  d.   Arterioskler.   Wiesbaden,   Bergmann,   1903. 
Klotz.  Adami  in   .Am.  J.   of  Med.   Sci..   Oct.,   1909.;  Jour.   Exp.   Med.,   1906,   p.  325. 
Knapp  Discussion  T.  O.  S.  XXIV.   1904.  p.   172. 
Leber,  Graefe-Saemisch.  V,  1,  p.  663. 
Magnus,   A.    f.    O.    LX,    1874.    p.    38. 
Mannhardt,   K.   Wochenschr.     f.   A.   XIII.  p.   132. 
Marchand,    Eulenberg's    Real    Encyclopedia. 

Marple.   Med.    Record,   Mar.    16.    1907;     Med.    Rec,    Mar.   11.   1893. 
Mirhaelsen,   C.   f.   p.   A.,   1899,   p.   106. 
Michel,    Lehrb.   der   Augk.,    H,   Aufl..   p.   447.      Zeitsch.    f.   A..   VTI,    p.   5.    Z.    f.    A.. 

XIII,  p.  -OS. 
Mott.    -Alllntt   and   PoUeston's    System,   Vol.    \\. 
Raehlmann,    Zeits,    f.    kl.    Med.   XVI,   5   u.    6,   p.    1.     Fortschr,   d.    Med.,    1899,   pp.   229- 

303.      Kl.   M.   f.   Augk.,   XXVII.  p.  242. 
Scbleich.   Mitteil,  aus   d'Oph.    Klinik  in   Tubingen    II,   2,   p    202. 
Scultet,  Ref.  in  Wilbrand  und  Saenger,  Vol.  IV,  p.  175. 
Seydel,   A.   f.  A.,  XXXVIII,  p.  157. 
Sidler-Huguenin   .\.  i.   A.   LI,   p.  27. 
Thoma,  A.  f.  O.   XXXV,  2.  p.  1. 
Tyson,   Brights  and   Diabetes. 

Virchow,   Uber  d.   Clinic  u.  aname  in  Gefasseaparat,   Berlin,   1872. 
Von   Graefe,   Angiektasie,   Leipzig,   1808,   p.   33. 
Wilbrand  und  Saenger,  Path.  d.'^Xetzhaut,  TV.  Wiesbaden,   1909,  p.  248. 

Stereogr.am  .32.  Retinal  Hemorrh.age  in  Arteriosclerosis. 
Right  eveground  of  a  man  47  years  of  age.  Works  in  a  brewery  where  he 
performs  heavy  manual  labor.  Consumes  a  great  quantity  of  beer  daily. 
P1ood  pressure,  190  mm.  Hg.  Urine  contains  albumin  and  casts,  but 
the  specific  gravity,  quantity  and  urea  content  do  not  indicate  advanced 
degeneration  of  the  kidneys.  Central  vision  In  the  right  eye  was  suddenly 
lost  about  one  month  ago.  In  the  left  eye  vision  is  normal.  Eye  tension  is 
not  unduly  increased.  Ovrhthalmoscopic  examination  reveals  extensive  de- 
generation of  the  retinal  vessels  in  both  eyes.     Right  eye: — Physiologic 


Fig.  69. 
Linear  Hemorrhage  in  Nerve  Fiber  Layer  r,f  Retina  (shown  at  a). 


^  Fig.  70. 

TTemorrha.ee';  involvinfj  the  Deeper  Layers  of  the  Retina. 


•  Figs.  71  and  72 — Hemorrhages  In- 
volving the  Layer  of  Rods  and  Cones, 
penetrating  the  External  Limiting 
Membrane  and  Tending  to  Spread  be- 
tween the  Retina  and  Choroid. 


Fig.   71. 


Fig.  73 — Small  Hemorrliagic  Extravasa- 
tion Elevating  but  not  Rupturing  the  In- 
ternal Limiting  Membrane  of  the  Retina, 
near  the  Macula. 


Fig-  7o 


SCLEROSIS    OF    KKIIXAL    VLSSELS llKMwK.wi.  v.,1..  71 

excavation  of  the  optic  disc,  but  no  neuritis.  I  he  macular  and  supra- 
temporal  regions  contain  numerous  small,  striate  hemorrhages.  Most  of 
the  extravasations  are  recent,  but  a  few  exhibit  yellowish-white  patches  of 
partial  absorption  and  degeneration.  The  bleeding  has  occurred  prin- 
cipally along  the  course  of  the  superior  temporal  vessels,  but  no  special 
point  of  closure  appears  in  the  vein.  No  exudates  are  seen  in  the  fundus. 
The  retinal  arteries  are  irregularly  narrowed  and  bordered  by  distinct, 
white  lines.  Several  small,  white  plaques  of  degeneration  appear  on  the 
surface  of  the  inferior  temporal  artery.  A  crossing  artery  indents  and 
obscures  an  underlying  vein  in  the  manner  characteristic  of  arteriosclerosis. 
The  veins  are  white  bordered,  tortuous  and  alternately  constricted  and 
dilated.  The  venous  terminals  are  unduly  prominent  and  very  tortuous, 
'f  he  inferior  temporal  veins  are  wider  at  the  periphery  than  at  the  disc. 
This  case  might  well  be  classed  as  one  of  hemorrhagic  albuminuric  retinitis. 

Inasmuch  as  bleeding  is  a  prominent  feature  in  most  of  the  retinal 
diseases  later  to  be  described,  the  text  will  be  better  understood  if,  at  this 
point,  the  subject  of  hemorrhage  is  somewhat  fully  discussed. 

Rktixal  Hemorrhage  is  one  of  the  most  common  pathologic  con- 
ditions observed  with  the  ophthalmoscope  and  occurs  as  a  symptom  in  a 
variety  of  Jocal  and  general  diseases.  The  amount  of  blood  extravasated 
varies  from  minute  extra^'asation  up  to  an  almost  continuous  sheet  covering 
the  fundus.  ExtensiAe  bleeding  breaks  down  all  barriers,  but  when  mod- 
erate its  position  in  the  retina  usually  can  be  determined  by  its  form. 
Thus,  in  the  nerve  fiber  layer  the  blood  follows  the  direction  of  the  fibers 
and  the  hemorrhages  appear  linear,  striped,  tongue-shaped  or  as  though 
splashed  on  with  a  brush  (Fig.  69).  In  the  deeper  layers  the  extrava- 
sations are  rounded  or  irregular,  their  form  being  determined  by  the 
perpendicular  fibers  of  Miiller  which  pre^^ent  the  blood  from  spreading 
(Fig.  70).  If  blood  penetrates  the  external  limiting  membrane  of  the 
retina  and  appears  among  the  rods  and  cones,  it  tends  to  spread  between 
the  choroid  and  retina  (Figs.  71-72).  Multiole  cauillary  hemorrhages 
consist  of  small,  round,  disseminated  flecks,  distributed  independently  of 
the  position  or  direction  of  the  retinal  vessels  (Stergm.  38).  Small, 
round  or  oval  extravasations,  which  lift  but  do  not  rupture  the  internal 
limiting  membrane,  occur  especially  in  the  macular  region  (Fig.  73). 
Allied  to  this  form  are  the  preretinal  hemorrhages.  If  hemorrhages  are 
very  large  or  the  bloo<i  Is  extravasated  with  rapidity  and  force,  it  enters 
the  vitreous.  Ble«d'-'i-  into  the  perivascular  sheaths  may  appear  as 
stripes  along  the  walls,  ci'  as  a  smooth  outlined  swelling  on  or  around 


72  THE    FUNDUS    OCULI. 

the  vessel.  These  hematomata  are  distinguishable  from  vascular  ectasiae 
by  their  dark  color  and  absence  of  axial  light  streak.  When  first  extrava- 
sated,  arterial  blood  may  be  lighter  in  color  than  venous  blood,  but  all 
hemorrhages  soon  darken.  The  color  of  the  blood  may  be  lighter  in  severe 
anemias,  but  this  is  of  little  value  in  diagnosticating  the  nature  of  retinal 
hemorrhages.  The  source  of  hemorrhage  is  more  readily  determined  by  its 
proximity  to  a  vessel  than  by  its  color;  but  even  this  test  is  unreliable, 
inasmuch  as  superficial  extravasations  may  change  position  or  blood  may 
be  drawn  from  a  distance  into  the  sheath  of  a  large  vessel,  or  a  large 
capillary  hemorrhage  may  occur  anywhere. 

The  size  of  a  vessel  does  not  determine  the  amount  of  blood  ex- 
travasated.  Bleeding  from  a  large  vessel  may  be  trivial,  while  in  retinal 
apoplexy  the  blood  comes  from  the  venules  and  capillaries. 

Retinal  hemorrhage  is  immediately  followed  by  resorption  of  the 
blood.  Small  surface  hemorrhages  fade  away  in  a  week  or  two,  clearing 
from  the  margin.  Large  hemorrhages  clear  from  both  periphery  and 
center.  White  patches  composed  of  phagocytic  leucocytes,  fatty  products 
of  degeneration  and  dead  retinal  elements  appear  in  and  around  the  ex- 
trav^asation.  The  blood,  fat  and  debris  are  removed  through  the  perivas- 
cular lymph  spaces  and  angle  of  filtration  in  the  anterior  chamber.  If  the 
retinal  vessels  are  sclerosed,  absorption  will  be  slow.  The  result  is  pro- 
longed action  of  the  decomposing  blood  toxins  upon  the  iris  and  pectinate 
ligament  and  this  may  excite  iritis  or  glaucoma.  When  hemorrhages  are 
small  and  confined  to  the  nerve  fiber  layer,  they  disappear,  leaving  no 
trace.  When  they  are  large  and  deeply  situated,  yellowish  or  white  dis- 
colorations  usually  remain.  W^hen  blood  has  penetrated  into  the  deep 
layers  of  the  retina,  tissue  destruction  is  excessive  and  the  blood  clot  is  re- 
placed by  a  shining,  white  cicatrix  or  by  a  mass  of  fibrous  tissue.  As  a 
rule,  little  or  no  pigmentation  follows  retinal  bleeding.  When  present,  it 
mav  be  either  heinatogenous  or  produced  by  massing  and  proliferation 
of  the  pigmented  epithelium.  In  some  cases  pigment  appears  on  the  disc 
after  absorption  of  blood. 

The  functional  disturbance  produced  bv  a  retinal  hemorrhage  depends 
upon  its  situation.  If  it  occurs  at  the  macula,  useful  vision  may  be  abolished 
(Fig.  72).  If  peripherally  situated,  it  may  create  so  little  disturbance  as 
to  escape  observation.  Moderate  bleeding  into  the  nerve  fiber  layer  does 
little  permanent  injury,  but  in  the  rod  and  cone  layer  it  is  destructive. 
Hemorrhage  into  the  vitreous  produces  cloudy  vision,  the  density  of  which 
depends  upon  the  amount  of  blood  in  front  of  the  retina. 


SCLEROSIS    OF    RRTIXAL    VESSELS M  I'.MORRHAGE.  73 

Under  any  circumstances,  spontaneous  retinal  hemorrhage  is  a  danger 
signal  of  the  first  importance.  Frequently,  the  first  sign  of  serious  disease 
is  a  single,  small  retinal  extravasation. 

ErioLOGv  OF  Retinal  Hemorrhage  in  General,  l^he  etiology 
of  spontaneous  retinal  bleeding  includes  all  diseases  or  conditions  which 
greatly  increase  or  diminish  the  blood  pressure,  retard  or  accelerate  the 
circulation,  induce  changes  in  the  heart  or  blood  vessels  or  modify 
the  composition  of  the  blood.  Foremost  are  degenerative  changes  in  the 
blood  vessels  which  occur  alone  or  as  the  prime  pathological  factor  in  other 
diseases. 

Arterios('Li:rosis.  According  to  Amman  bleeding  at  the  macula 
is  common  in  senile  arteriosclerosis.  Occasionally,  the  ophthalmoscopic 
picture  of  an  anglopathic  retinitis  consisting  of  exudation  and  hemorrhage 
may  develop  in  uncomplicated  angiosclerosis.  Straub  followed  for  six 
years  the  courses  of  15  cases  of  retinal  hemorrhage  from  arteriosclerosis. 
The  bleeding  exhibited  a  strong  disposition  to  occur  in  the  left  eye. 
Straub  states  that  the  prognosis  for  life  is  not  so  bad  in  the  young  and 
very  old  as  it  is  in  people  of  middle  life.  I'or  example,  nine  of  the  cases 
were  between  the  ages  of  45  and  65  years.  Of  these,  six  died  within 
six  years,  five  of  them  from  apoplexy.  Derby  observed  90  cases  of  retinal 
hemorrhage  in  people  over  40  years  of  age.  Of  these  he  ascertained  the 
death  of  31,  of  which  25  died  within  two  years;  14  from  apoplexy  and  1  1 
from  heart  disease.  Hemorrhage  from  the  retinal  vessels  is  not  un- 
common in  degenerated  eyes.  I  have  examined  microscopically  a  number 
of  such  bulbs  in  which  excessive  hemorrhage  had  occurred,  either  sponta- 
neously or  from  slight  injury. 

Retinal  bleeding  usually  depends  upon  degeneration  of  the  blood  ves- 
sels. The  blood  may  escape  either  bv  rhexis  or  diapedesis.  Harms  does 
not  accept  the  view  that  the  sclerosed  parts  of  a  vessel  tend  to  bleed,  al- 
though he  admits  that  they  easily  rupture  from  sudden  fall  of  intraocular 
pressure,  as  m  opening  the  anterior  chamber.  A  sclerosed  wall  offers 
greater  resistance  to  gradually  developeci  pressure  than  a  normal  wall,  and 
diai:edesls  occurs  less  readily.  The  unyielding  character  of  sclerosed 
vessels  is  demonstrated  by  the  following  experiments  of  Thoma  on 
n.davers.  In  an  arteriosclerotic  body,  four  liters  of  salt  solution,  injected 
into  the  blood  vessels,  produced  edema  of  the  lower  extremities,  while  in 
non-sclerotic  subjects  seventeen  liters  were  injected  before  edema  appeared. 
A  much  longer  time  was  required  to  inject  a  given  amount  of  fluid  into  an 
arteriosclerotic  subject  than  into  one  with  normal  vessels. 


74  THE    FUNDUS    OCULI. 

In  leukemia  and  permc'wiis  anemia  hemorrhages  into  the  retina  occur 
in  nearly  all  cases.  In  leukemia  fresh  extravasations  often  contain  white 
centers.  Similar  hemorrhages  occur  In  pernicious  anemia  but  it  is  not 
certain  that  the  white  centers  appear  at  the  time  the  blood  is  extravasated. 
The  peculiarities  of  leukemic  hemorrhages  are  considered  In  connection 
with  the  retinitis  that  occurs  in  that  disease   (Stergm.  39). 

Anemia.  Retinal  hemorrhage  is  a  doubtful  event  In,  so-called,  simple 
anemia.  Anemias  always  are  secondary,  therefore,  associated  retinal 
hemorrhage  should  be  classed  as  a  symptom  of  the  underlying  disease. 
Pick  found  retinal  hemorrhages  In  30  per  cent  of  the  intense  anemias  pro- 
duced by  carcinomatosis.*  The  anemic  condition  may  be  responsible  for 
retinal  bleeding  which  occurs  in  cases  of  Intestinal  parasites, t  tuberculosis, 
exhausting  hemorrhage,  etc. 

Chlorosis.  In  chlorosis  as  In  simple  anemia,  retinal  bleeding  is 
infrequent.  When  It  does  occur  other  complicat'ons  usually  coexist,  i.  e., 
disordered  menses,  or  choked  disc.  Kaufmann  frequently  has  found  fatty 
degeneration  of  the  Intima  In  the  blood  vessels  of  chlorotlcs.  Thrombosis, 
as  a  rare  event,  belongs  to  the  history  of  chlorosis,  \elther  retinal  hemor- 
rhaQ;e  nor  optic  neuritis  should  be  attributed  either  to  anemia  or  chlorosis 
until  all  other  possible  causes  have  been  eliminated.  It  Is  a  point  of  diag- 
nostic importance  that  In  pernicious  anemia  hemorrhage  into  the  retina 
occurs  In  all  cases,  while  in  simple  anemia  and  chlorosis  it  is  extremely  rare. 

Malaria.  Malaria  appears  to  be  a  frequent  cause  of  retinal  bleeding 
i'l  t'''ose  regions  where  the  disease  is  severe  in  type.  The  hemorrhage  may 
be  slight  or  so  extensive  as  to  appear  in  the  vitreous.  One  or  both  eyes  may 
be  affected.  It  may  occur  In  the  first  week  during  the  fever  or  in  the  late, 
cachectic  stage,  at  which  time  vascular  disease  may  exist.  In  ten  cases 
that  died  from  pernicious  and  comatose  forms  of  malaria,  Guarnleri 
regularly  found  stasis,  edema  and  hemorrhage  in  both  brain  and  retina. 
In  the  capillaries  the  red  blood  corpuscles  contained  plasmodia  and  pig- 
ment and  the  white  cells  enclosed  pigment. 

Syphilis.  Simple  retinal  hemorrhage  is  uncommon  in  syphilis.  When 
present  it  generally  occurs  in  connection  with  papillitis,  retinitis  or  local 
vascular  disease. 

Stasis.  When  the  retinal  vessels  are  sound  they  withstand  a  high 
degree  of  distension  without  loss  of  blood.     This  is  well  illustrated  in  cases 


*A1so  Stephen  Mackenzie. 

tAnkylostonnim  duodenale   (Nieden,  Fisher  and  others.)     Botlirioceplialous  latus 
(Njitanson  and  others). 


SCLEROSIS    OF    KlillXAL    VESSELS HEMORRHAGE.  75 

of  retinal  cyanosis  (Stergm.  58),  'i  which  bleeding  occurs  only  in  late  stages 
after  vascular  degeneration  has  ensued.  Cases  are  reported  of  retinal 
hemorrhage  due  to  venous  congestion  produced  by  paroxysms  of  whoop- 
ing cough*  and  epilepsy.!  In  these  and  similar  cases,  vessel  alterations 
cannot  be  excluded  simply  because  they  are  unrecognizable  with  the  oph- 
thalmoscope. It  is  doubtful  if  any  degree  of  muscular  exertion  produces 
sufficient  congestion  to  cause  retinal  hemorrhage.  I  have  microscopically 
examined  the  eyes  of  six  healthy  adults  killed  by  the  application  of  a  pow- 
erful electric  current,  but  in  none  of  them  did  I  discover  retinal  bleeding, 
although  by  this  method  of  death  all  the  voluntary  muscles  are  thrown 
into  violent  contraction.  A  condition  known  as  traumatic  asphyxia  ensues 
when  the  body,  especially  the  thorax,  is  subjected  to  great  external  pressure. 
In  this  condition  bleeding  into  the  retina  and  optic  nerve  may  occur, 
sometimes  several  days  after  the  compression. 

Natal  Retinal  Hemorrhage  is  produced  in  a  somewhat  similar 
manner  from  compression  of  the  head  and  thorax  during  birth.  Possibly, 
attempts  to  breathe  while  the  body  is  yet  in  the  parturient  canal  may  induce 
sufficient  turgescence  to  cause  retinal  bleeding.  These  hemorrhages  are 
said  to  occur  in  nearly  all  cases  of  protracted  and  complicated  labor  and 
in  feeble  children.  Thus,  Coburn  in  37  stillborn  and  short-lived  infants, 
found  retinal  hemorrhages  in  17.  Natal  hemorrhages  usually  disappear 
leaving  no  trace  of  their  existence  in  the  retina.  Naumhoff  suggests  that, 
occurring  in  the  macula,  they  are  the  usual  cause  of  congenital  amblyopia. 
Local  stasis  from  obstructed  venous  outflow  is  the  principal  cause  of 
hemorrhage  in  choked  disc,  thrombosis  of  the  central  vein  and  compression 
of  the  nerve.  Sudden  local  congestion  may  be  the  determining  cause  of 
bleeding  from  diseased  retinal  vessels,  as  when  the  aqueous  is  ev^acuated 
in  operations  for  glaucoma.  Besides  the  diseases  already  mentioned, 
hemorrhages  in  the  retina  have  been  reported  as  occurring  in  influenza, 
typhus,  typhoid,  plague,  erysipelas,  smallpox,  measles,  scarlet  fever,  gout, 
septic  retinitis,  Weil's  disease,  childbirth,  climacteric,  intestinal  disorders, 
menstrual  disturbance,  masturbation,  sexual  excesses,  etc.  Retinal  hemor- 
rhage is  a  rare  and  inconspicuous  event  in  ocular  disturbances  produced  by 
poisons.  When  bleeding  occurs  it  appears  to  be  associated  with  retinal 
and  nerve  congestion,  indicating  that  it  is  due  to  changes  in  the  circulation 
and  walls  of  the  vessels  rather  than  alterations  in  the  composition  of  the 
blood.      Poisoning  by  coal  gas,  snake  venom,   nitrobenzol,   analin,   phos- 


*  Landesburg,  Teillais.  f  de  Gom  ea. 


76  IHE    FUNDTS    OCULI. 

phorous,  sulphuric  acid,  felix  mas  and  alcohol  are  described  as  having 
produced  extravasations  of  blood  in  the  retina,  Wagenmann  described 
a  case  of  extensive  skin  burn  in  which  multiple,  small  retinal  hemorrhages 
appeared  in  both  eyes.  They  were  attributed  to  the  same  blood  changes 
which  after  severe  burns  produce  ulcerations  in  the  bowels. 

There  are  two  forms  of  intraocular  bleeding  which  present  features 
requiring  extended  descriptions.  These  are,  preret'inal  hemorrhage  and 
intravitreoiis  hemorrhage. 

Preretinai.  or  Sub  HYALOID  Hemorrhage  appears  in  the  form  of  a 
large,  thin,  discoid  clot  on  the  surface  of  the  retina.  After  a  few  days 
the  blood  corpuscles  separate  and  form  a  lower,  dark-red  part,  divided 
by  a  sharp,  horizontal  line  from  an  upper,  pale-red  part  composed  of 
plasma.  The  plasma  is  resorbed  before  the  corpuscles,  leaving  a  slight 
opacity.  Instead  of  being  resorbed  the  blood  may  enter  the  vitreous. 
Griffiths  has  seen  the  vitreous  suddenly  fill  with  a  broken-down  blood  clot 
from  yielding  of  the  structures  in  front  of  a  subhyaloid  hemorrhage.  It  is 
generally  assumed  that  these  hemorrhages  are  situateci  between  the  retina 
and  vitreous.  Were  this  true  it  might  be  expected  that  the  blood  would  be 
diffusely  distributed  and  gravitate  to  the  bottom  of  the  globe;  whereas, 
the  clots  are  sharply  circumscribed,  present  great  uniformity  in  shape  and 
manifest  little  tendency  to  change  their  position.  These  characteristics 
render  it  probable  that  the  blood  is  immediately  beneath  the  internal 
fimiting  membrane  of  the  retina.  Probably,  the  clouded  appearance  which 
remains  after  resorption  of  the  plasma  is  an  opacity  in  the  retina.  A  pre- 
retinai hemorrhage  does  not  injure  the  percepti^'e  tissue  of  the  retina; 
conseiuently,  after  removal  of  the  blood,  vision  will  be  completely  restored. 

Stereogram  .33.  Preretinai  (Subhyaloid)  Hemorrhage. 
Woman  29  years  of  age.  She  is  an  ill-nourished,  sweat-shop  seamstress. 
About  one  month  ago,  while  bending  over  her  work,  a  cloud  suddenly 
apDeared  before  the  left  eye  which  completely  destroyed  central  vision. 
With  the  ophthalmoscope,  an  enormous  extravasation  of  blood  is  seen 
co\ering  the  entire  macular  region.  It  lies  on  the  surface  of  the  retina  in 
front  of  the  vessels,  is  slightly  elevated  and  its  outline  is  sharply  defined. 
The  lower  portion  of  the  hemorrhage  consists  of  a  dark-red  clot  and  the 
upper  portion  of  very  light-red  plasma.  The  retinal  vessels  present  no 
positive  evidence  of  arteriosclerosis.  Physical  examination  fails  to  dem- 
onstrate tuberculosis  or  syphilis. 

At  the  time  this  case  came  under  observation  tuberculin  was  not  em- 
ployed for  diagnostic  purposes. 


SCLKKOSIS    OF    RETINAL    VESSELS HEMORRHAGE.  77 

Intravitreous  Hemorrilage.  Blood  In  the  vitreous  appears  oph- 
thalmoscopically  as  slightly  movable  Hocculi  or  ill-defined  masses;  some- 
times as  a  large  clot  the  surface  of  which  may  undulate  on  movement  of 
the  eye.  These  hemorrhages  appear  black  in  color  or  give  a  reddish 
gleam.  When  situated  far  forward  in  the  vitreous  and  the  pupil  is  widely 
dilated,  the  effusion  may  be  recognized  as  blood  by  oblique  illumination. 
By  transillumination,  fresh  blood  is  transparent  but  clotted  blood  casts  a 
shadow.  Sometimes  the  iris  is  colored  brown  or  green  from  absorption  of 
blood  pigments.  Months  usually  elapse  before  resorption  of  a  vitreous 
clot  is  completed.  The  blood  may  color  the  \itreous  and  cause  red 
vision,  which  later  becomes  yellow  or  green.  Tuchs  has  obser\ed  in  some 
cases  of  traumatic  vitreous  hemorrhage,  some  time  after  the  injury,  the 
coloring  matter  of  the  blood  clot  suddenly  dissohe  and  stain  the  ocular 
fluids.  In  such  cases  the  aqueous  is  colored  red  antl  the  iris  appears  as 
though  seen  through  ruby  glass.  \A'hen  hemorrhage  has  been  large  and 
the  vitreous  badly  disrupted,  permanent  opacities  may  remain  or  mem- 
branes of  dense  connective  tissue  may  form  which  seriously  impair  vision 
(Stergtii.   61  ) . 

In  profuse  retinal  hemorrhage  from  anv  cause,  blood  may  enter 
the  vitreous.  Aside  from  such  mciciental  cases,  a  special  form  occurs 
known  as  recurrent  intrnvitreous  hcmorrhayc  in  sonny  people.  \on 
Graefe  first  separated  this  class  of  cases  in  which  vision  is  periodically 
lost  from  recurring  hemorrhages  into  the  vitreous.  Subsequent  observa- 
tions of  this  affection  have  added  to  our  knowledge  of  its  clinical  features 
but  have  done  little  to  clear  up  its  etiology  and  pathology.  It  occurs  in 
young  people,  chiefly  males.  Suddenly,  without  apparent  cause,  vision  of 
the  affected  eye  is  lost  from  effusion  of  blood  into  the  vitreous.  As  a  rule, 
the  amount  of  blood  extravasated  is  sufficient  to  obscure  the  eyeground. 
Exceptionally,  blood  appears  in  the  anterior  chamber.  Resorption  quickly 
ensues,  but  after  a  variable  period  hemorrhage  recurs  and  vision  again  is 
lost.  The  attacks  may  be  few  in  number  or  repeated  until  eyesight  Is 
destroyed.  As  in  other  forms  of  intraocular  hemorrhage,  iritis  or  glau- 
coma may  result.  Many  cases  give  a  history  of  recurring  epistaxis  which 
ceases  after  the  advent  of  intravitreous  bleeding.  The  hemorrhages  usually 
appear  first  between  the  fifteenth  and  twenty-fifth  years,  very  rarely 
after  the  fortieth  year.  One  or  both  eyes  may  be  affected.  When  uni- 
lateral, a  marked  predilection  is  shown  for  the  left  side.  Both  eyes 
may  be  affected  simultaneously  or  at  different  times.     The  mere  presence 


78  THE    FUNDUS    OCULI. 

of  blood  in  the  vitreous  does  not  increase  intraocular  tension,*  and  in 
some  cases  tension  is  diminished. t  The  interval  between  hemorrhages 
varies  from  a  few  weeks  to  months.  In  rare  cases,  which  may  belong  to  a 
different  class,  years  elapse  between  attacks. |  Considering  that  the  vit- 
reous contains  no  blood  vessels,  resorption  in  this  form  of  bleeding  is 
rapici,  two  to  four  months  being  the  usual  period.  A  remarkable  case  is  re- 
ported by  Jacquesu  in  which  the  hemorrhage  was  removed  in  three  days. 
In  this  case  it  is  probable  that  the  blood  came  from  the  ciliary  body  and 
lay  between  the  lens  and  vitreous.  Exceptionally,  resorption  is  delayed 
for  a  year  or  longer. §  The  predominance  of  male  over  female  cases 
is  shown  by  Wilbrand  and  Saenger,  who  collected  73  typical  cases  of  this 
affection  of  which  IS  were  females  and  58  males. jj  Among  females  it 
is  possible  that  the  periodic  loss  of  blood  from  menstruation  may  di- 
minish the  tendency  to  intraocular  hemorrhage. 

The  source  of  the  hemorrhage  is  a  matter  of  doubt.  Probably  it 
is  not  the  same  in  all  cases.  The  occasional  association  of  changes  in 
the  choroid  has  led  many  authors  to  regard  this  membrane  as  responsible 
for  the  bleeding. H^  In  the  few  microscopic  examinations  which  have  been 
made  in  cases  of  recurrent  intravitreous  bleeding,  vascular  changes  have 
been  reported.**  A  combined  disease  of  the  choroid  and  retina  is  asr 
sumed  bv  Leber  to  be  responsible  for  these  hemorrhages.  Michel  makes 
the  important  statement  that  a  vitreous  hemorrhage  often  is  the  first 
symptom  of  tuberculosis  oi  the  ciliary  body.  I  have  seen  one  case  in  which 
the  bleeding  was  due  to  a  small  tubercular  lesion,  situated  at  the  extreme 
anterior  limit  of  the  ophthalmoscopic  field.  The  uniformity  of  symptoms 
exhibited  by  cases  of  this  affection  indicates  a  common  cause,  regardless 
of  the  source  of  bleeding.  Axenfeld  and  Stock  affirm  that  hemorrhage 
due  to  tuberculosis  of  the  vessel  walls  may  occur  without  evident  lesion 
of  the  iris  or  choroid.  If  this  is  true,  it  explains  how  blood  may 
come  from  the  ciliarv  body,  choroid  or  retina  without  giving 
>Msible  evidence  of  its  origin.  The  characteristic,  sudden,  abundant  hemor- 
rhage that  occurs,  suggests  rupture  of  a  single,  large  vessel,  differing 
from  the  slow  bleeding  of  hemorrhagic  retinitis,  in  which  a  lake  of  blood 
may  cover  the  fundus  without  escaping  into  the  vitreous    (Stergm.   46). 

*Zieminski.  five  cases  in  whicli   tension   was  unaffected. 

tXieden,  tension   diminished. 

JBenin.a:.  interval   11   years.      Kipp,  interxal   fi  years. 

§Mayweg.  18  months 

|'Benin£T,  217   cases;    females   74;     males   14?.. 

HXieden.   Fehr.    Knapp. 

**SchefTfe].  four  cases.      Fehr.  three  cases. 


SCLF'.ROSIS    OF    RFyilNAL    VESSELS HEMORRHAGE.  79 

From  the  evidence  at  hand,  it  would  appear  that  recurrent  intravitreous 
hemorrhage  is  due  to  some  process,  probably  tubercular,  which  erodes  a 
large  vessel  in  the  ciliary  body,  retina  or  choroid. 

Other  diseases  which  have  occurred  in  connection  with  intravitreous 
hemorrhage  and,  presumably  were  causati\-e,  are  hereditary  and  acquired 
synhilis,  gout,  menstrual  diseases,  anemia,  exhaustion,  malaria,  endogenous 
infection,  constipation,  hypertrophy  of  the  heart,  hemophilia,  purpura 
and  scur\-y.  When  the  tendency  to  hemorrhage  already  exists,  its  im- 
mediate cause  may  be  congestion  due  to  stooping,  sneezing,  etc. 

The  prognosis  of  intravitreous  bleeding  appears  to  be  more  favorable 
in  the  young  than  in  those  of  advanced  years.  If  iritis  develops  it 
may  subside  with  disappearance  of  the  blood,  but  it  is  a  serious  compli- 
cation and,  if  in  addition  eye  tension  is  increased,  the  eye  will  probably 
be  dest roved  by  glaucoma. 

B'lhl'ioc/rcipliy. 

Amman,  Beitrag.  /..  p.  Aiigke.  XXXVIII.,  p.  1. 

Beninq,   inaiu>.   Uis.  Giessen.   ]'.)()(). 

Coburn,   A.  of  Oph.   1904.  XXX  II  I.   p.  SoG. 

De  Gouvca,  Anna!.  d'Ocul.  CXVIII,  p.  9fr. 

Derby,    Mass.    Med.    .Soc,    lune    S.    lUOT. 

Fchr,  C.  f.  p.  A.  XXV.  2. 

Fisher,   C.   f.   p.   .A    1S9().  p.  lUid. 

Fncbs,  :!d   .\mmer.    H)()S.   p.   470. 

Griffiths,    .\orris    &    Oliver,    Vol.    :j.   p.    :?8:r 

Gnarnieri,  Arch.  p.   1.   s.  med.,   XXI,  p.   1. 

Harms,  A.  f.  O    XXX TV,  lOO.',   &  LXI.   1905.     Corespbl.  f.   Schw.  Artz,  XXVIII, 

1908.  p.  :!:!8. 
Jacqnesn,   Oph.    Klin.,   1^99,   XXII. 
Jarnatowski,  Recueil.  d'C,  p.  .tTT. 
Kaufmann,  Lehrb.  d.  sp.  path.  Anat.,  p.   4."). 
Kipp,  Trans.   A.   Oph.   S.   1895,  p.  4:^5. 
Landesburg.   Med.   &  .Surg.   Rep.   XLIII,  Sept. 
Lawford,  Oph.  Rev.   1893,  p.  347. 
Ma\\veg,   Herich  n.  d.  Oph.  Gel.  z.  Heidg.,  p.  92. 
Natanson.    lahreber.    f.   Oph.    1894.   p.   528. 
Naumhoff.   A.  f.  O    XXXVI,   1890,  p.  180. 

Nieden,  14  Sitz.  d.  Heidg.  Oph.  Gesel.  p.  8:  Cent.  f.  p.  .\.  1903.  p.  ?,f,(\. 
Pick,    Klin.    Monsb.    f.    A..   XXXIX.    p.    177. 
Schefifels,  Dentsch.  Med.  Wochensft.  1897,  XIII. 
Straub,   Klin.   M.  f.  A.  XLVI,   1908,  p.   517. 
Teillias,  Rec.   d'O.   1895. 

Thoma.   quoted  by  Mott.  Allbutt   &  Rolleston.  vol.   VI. 
Von  Graefe,  A.  f.  O.,  vol.  1. 
Wagenmann,  A.  f.  A.  XXXIV,  2,  p.   111. 

Welch,  Trans.  Ass.  Am.  Phys.  1897,  p.  121.  Allbutt  &  Rolleston,  1909,  vol.  \T.  p.  762. 
Wilbrand   k  Saenger,  Path.  d.   Netz.  1909.  p.  215 
Zieminski,   Rec.   d'O.   1887,  p.   709. 


Chaptkr  VI. 
ANGIOPATHIC   RETINITIS. 

Simple  sclerosis  of  the  retinal  vessels  is  never  a  local  disease,  and  the 
slightest  pathologic  change  in  the  vessel  walls  is  positive  evidence  that 
wide-spread  vascular  disease  is  imminent  or  alreaciy  established. 

Ophthalmoscopic  evidence  of  angiosclerosis  varies  from  mere  tor- 
tuosity of  vessels  to  destruction  of  the  retina  by  hemorrhage,  exudation 
or  degeneration.  The  intimate  relation  between  degeneration  of  the 
blood  vessels  anti  chronic  inflammation  of  the  inner  layers  of  the  retina 
has  led  to  the  belief  that  symptomatic  retinitis,  as  it  occurs  in  different 
diseases,  is  only  an  expression  of  disturbed  circulation.*  The  familiar 
ophthalmoscopic  picture  of  "  Bright's  "  disease,  formerly  supposed  to 
characterize  that  affection,  has  been  known  to  occur  without  albuminuria 
as  the  result  of  infection  in  scarlatina,  measles,  erysipelas,  malaria,  anchy- 
lostoma  and  syphilis.  Typical  examples  ha^■e  been  reported  also  in  diabetes, 
scurv'y,  rer-iicious  anemia,  carcinomatosis,  brain  tumor,  in  poisoning  by 
lead  and  felix  mas;  and  in  the  absence  of  known  toxins  or  infection,  in 
uncomplicated  arteriosclerosis.  Wilbrand  and  Saenger,  and  Parsons  have 
each  reported  cases  exhibiting  a  "  macular  star  "  for  which  no  cause  could 
be  found.  While  the  fundus  changes  in  any  case  of  symptomatic  retinitis 
may  strongly  suggest  the  nature  of  the  general  disease,  they  cannot  be 
considered  as  strictly  diagnostic.  Therefore,  it  is  inaccurate  to  describe 
special  forms  of  retinitis  as  characteristic  of  a  particular  disease.  To 
meet  a  difficulty  in  terminology,  Wilbrand  and  Saenger  designate  all 
inflammations  of  the  inner  retinal  layers  ''  angicpath'ic  rethiitis  "  and  qualify 
this  term  bv  the  name  oi  the  associated  disease,  as  "  albuminuric  angio- 
pathic  retinitis,"  "  diabetic  angiopathic  retinitis,"  etc.  This  nomen- 
clature may  be  not  wholly  free  from  criticism,  but  it  possesses  the  great 
advantage  of  convenience.  Although  the  appearance  of  an  angiopathic 
retinitis   is   not   always  diagnostic,    it   usually   suggests   the   nature   of   the 


*A  theory  has  lieen  advanced  that  both  vascuhTr  and  tissue  defeneration  are 
(aured  by  the  local  action  of  some  toxin.  In  the  retina,  tissue'  defeneration  appears 
to  be   secondary  to  mechanical  closure  of  vessels. 

80 


ANGIOPATHR'    KKTIXHIS.  81 

general  disease.  Therefore,  from  a  clinical  standpoint  it  is  desirable  to 
separate  and  describe  angiopathic  retinitis  as  it  usually  appears  when 
associated  with  certain  diseases. 

Angiopathic  Albuminuric  Retinitis.  A  distinction  is  drawn  be- 
tween the  acute  and  chronic  forms  of  retinitis  occurring  in  albuminuria. 
The  acute  type  occurs  in  acute  diffuse  nephritis,  in  which  a  stage  of  retinal 
hyperemia  and  exudation  often  is  followed  by  recovery.  In  the  chronic 
type  the  stage  of  hyperemia  almost  invariably  passes  into  that  of  de- 
generation. These  two  forms  are  known  also  as  the  exttdat'rce  and  the 
degenernthe. 

In  the  great  majority  of  cases  angiopathic  retinitis  occurs  in  con- 
nection with  chronic  nephritis.  Ihe  ophthalmoscopic  picture  may  present- 
any  pathologic  condition  of  the  retina  resulting  from  vascular  degeneration. 
Clinically,  however,  the  term  "  albuminuric  retinitis  "  has  been  applied  to 
a  group  of  symptoms  consisting  of  venous  distension,  mild  neuritis,  retinal 
opacity,  exudation,  hemorrhage,  retinal  degeneration  and  atrophy.  Any 
or  all  of  these  changes  may  exist  in  a  given  case.  The  features  presented 
by  the  average  case  of  angiopathic  retinitis  are  best  described  by  dividing 
the  disease  into  the  following  four  stages:  (1)  Precursory  or  Prealbu- 
n.Inurlc;  (2)  Hyperemic  or  Congestive;  (3)  Degenerative;  (4)  Atrophic. 

SlERKOCIRAM   34.       PRECURSORY   StAGE   OF   AnGIOPATHIC    RETINITIS. 

Left  fundus  oculi  of  a  laborer  S?>  years  of  age.  Addicted  to  the 
'mmoderate  use  of  alcohol  and  tobacco.  Blood  pressure  190  mm.  Hg. 
The  urine  contains  neither  albumin  nor  casts,  but  the  percentage  of  urea 
is  low.  Has  persistent,  dull  headache.  The  fundus  conditions  are  similar 
in  each  eye.  The  optic  disc  has  a  brick-red  color.  The  retinal  arteries 
pursue  a  straight  course.  Their  axial  light  streak  is  very  bright  and  well 
defined,  producing  the  effect  known  as  "  silver  wire  arteries."  The  walls 
of  the  arteries  are  not  visible,  but  that  they  are  sclerosed  is  demonstrated 
by  the  manner  in  which  they  indent  underlying  veins.  The  larger  veins 
are  widened  and  tortuous  and  their  light  streaks  are  pronounced.  The 
small  vessels  around  the  disc  and  the  small  veins  throughout  the  fundus  are 
unduly  distinct  and  their  terminals  twisted. 

The  class  of  fundus  cases  here  defined  as  precursory,  or  prealbu- 
minuric  retinitis  are  only  examples  of  retinal  arteriosclerosis  which  have 
just  entered  the  stage  of  arterial  rigidity  and  venous  dilation.  The  systemic 
anglosclerosis  which  Invariably  attends  these  retinal  changes  usually  re- 
sults in  chronic  nephritis.  In  a  certain  number  of  cases,  however,  vas- 
cular degeneration   and   Its  sequelae  are   farthest  advanced   In  the   brain, 

G 


82  THE    FUNDUS    OCULI. 

heart  or  other  organs,  and  the  focus  of  disease  is  situated  elsewhere  than 
in  the  kidneys.  Of  all  phases  presented  by  angiopathic  retinitis,  this 
is  the  most  important  to  recognize.  The  spectacular  fundus  pictures 
of  the  later  stages  of  this  disease  indicate  that  irreparable  destruction  has 
already  occurred  and  a  fatal  termination  is  impending;  whereas,  if  the 
slight  but  significant  alterations  of  the  precursory  stage  are  discovered, 
the  progress  of  disease  may  be  arrested  or,  at  least,  delayed.  The  im- 
portant retinal  vascular  changes  of  the  precursory  stage  frequently  escape 
observation.  They  are  not  prominent  and  must  be  diligently  sought  with 
the  ophthalmoscope  through  a  wide  pupil. 

The  effect  of  heightened  blood  pressure  on  the  retinal  vessels  is  mani- 
fested by  the  veins  which  become  visibly  distended  and  tortuous.  The 
condition  of  the  terminal  venous  twigs  is  of  especial  diagnostic  significance. 
They  present  a  curly,  "  corkscrew  "  appearance,  described  also  a» 
"  crinkled  "  by  Alleman.  These  little  vessels  should  always  be  looked 
for  around  the  macula  and  disc.  De  Schweinitz  calls  attention  particularly 
to  one  of  them,  frequently  seen  descending  from  a  transverse  macular 
artery.  The  venous  distension  which  exists  in  established  arteriosclerosis 
extends  to  the  capillaries  on  the  disc,  and  the  latter  assumes  a  dirty,  brick- 
red  color.  Papillitis  may  develop  in  the  absence  of  albuminuria,  from 
uncomplicated  vascular  degeneration   (Stergm.  31). 

Diagnosis.  A  fundus  exhibiting  the  features  of  established  angio- 
sclerosis  is  "  prealbuminuric  "  in  that  this  condition  frequently  antedates 
the  development  of  nephritis  and  always  precedes  albuminuric  retinitis. 
The  case  presented  above  is  typical,  with  straight  arteries  and  tortuous 
veins.  In  another  case  it  may  happen  that  the  arteries  have  retained  more 
or  less  of  the  tortuosity  acquired  in  the  stage  of  arterial  relaxation  and 
dilation.  The  characteristic  indentation  and  obscuration  of  a  vein  by  an 
overcrossing  artery  are  positive  ophthalmoscopic  proof  of  retinal  arterio- 
sclerosis. Next  in  diagnostic  importance  are  crinkled  v^enous  terminals 
and  a  strong  axial  light  streak  on  the  arteries.  In  a  doubtful  case,  a  retinal 
hemorrhage,  however  trifling,  or  an  opaque  speck  on  an  artery  would  be 
conclusive  evidence  of  angiopathic  disease.  In  hypermetropia  the  disc 
may  be  red  and  the  vessels  tortuous,  but  there  is  no  vascular  degeneration. 

Developed  Retinitis.  Symptoms.  In  nearly  all  cases  of  angio- 
pathic albuminuric  retinitis  both  eyes  are  affected.*     Frequently,  the  two 


*Va^cular  degeneration   probably  exists   in  both   eyes  in  all   cases   of  retinitis  of 
albuminuria. 


ANCJIUFATHIC    rktinhis.  83 

eyes  are  attacked  at  different  times  and  in  varying  degree.  Moreover, 
the  manifestations  of  the  vascular  disease  may  dilfer  widely  in  each  eye. 
Thus,  in  case  of  stereogram  43  typical  angiopathic  retinitis  existed  in  the 
right  eye  and  closure  of  the  central  artery  in  the  left.  All  the  external 
ocular  manifestations  of  arteriosclerosis  may  appear  in  albuminuria, 
especially  ecchymoses  and  fugitive  edema  of  the  eyelids.  Wharton 
Jones  and  Schobl  haAe  seen  hemorrhage  into  Tenon's  capsule.  Post- 
hemorrhagic glaucoma  may  dexelop,  but  spontaneous  glaucoma  is  unusual, 
notwithstanding  the  fact  that,  in  chronic  albuminuria,  degeneration  of  the 
blood  \essels  is  extreme.  No  connection  has  been  traced  between  albumin- 
uria and  development  ot  cataract.  Detachment  of  the  retina  is  common 
in  the  late  stages  of  chronic  nephritis,  especially  it  there  is  anasarca.  In 
chronic  nephritis  the  \essels  of  the  choroid  undergo  degenerations  anal- 
ogous to  those  occurring  in  the  retina,  but  the  disastrous  results  of  retina! 
arteriosclerosis  usually  are  a\erted  in  the  choroid  by  the  free  anastomoses 
existing  between  its  \essels.  It  is  said,  however,  that  chorioretinitis  of 
angiopathic  origin  can  develop.  The  presence  of  this  complication  would 
be  recognized  by  pigment  heaped  around  white  plaques  of  degeneration  and 
by  disturbance  of  the  retinal  epithelium  and  pigment  wandering.* 

The  degree  of  functional  eye  disturbance  experienced  in  the  retinitis 
of  albuminuria  depends  upon  the  nature  and  location  of  the  lesion  rather 
than  upon  the  severity  of  the  retinal  inflammation  or  gravity  of  the  general 
disease.  Probably,  visual  acuity  always  is  blunted  even  though  it  may 
not  be  indicated  by  the  ordinary  card  tests.  At  first,  there  is  complaint  of 
a  slight  fog  before  the  eyes,  an  effect  which  is  produced  by  the  presence  of 
retinal  opacity  before  the  percipient  layers  of  the  retina.  Lesions  situated 
in  the  macula  or  detachment  of  the  retina  produce  great  impairment  of 
vision.  Hemorrhages  affect  sight  according  to  their  situation  and  extent. 
Total  loss  of  vision  seldom,  if  e\er,  is  due  to  uncomplicated  retinitis  or 
subsequent  atrophy.  Sudden  blindness  in  both  eyes  means  uremic  amau- 
rosis. Occurring  in  one  eye  it  may  indicate  either  retinal  detachment  or 
closure  of  the  central  artery.  The  visual  fields  usually  correspond  to  the 
destruction  wrought  in  the  retina,  differing  in  this  respect  from  diabetes  in 
which  amblyopias  with  scotomata  occasionally  occur.  Concentric  limitation 
of  the  fields,  described  by  Schlesinger,  and  paracentral  ring  scotoma  by 
Leber,  have  not  been  accepted  as  belonging  to  the  history  of  the  disease.' 
Color  scotomata  have  been  described  by  Simon  and  others  as  characteristic 


*The  condition  is  so  rare  that  complications  probably  exist. 


84  THE    FUNDUS    OCULI. 

of  albuminuria,  a  conclusion  which  has  not  been  confirmed.  These  scoto- 
mata  present  wide  variations  and  probably  depend  upon  macular  changes. 
Cerebral  hemorrhage  is  not  uncommon  in  albuminuria,  and  hemianopsia  or 
other  central  disturbance  of  vision  may  ensue.  Ophthalmoscopically  visible 
changes  exist  long  before  vision  is  affected  or  albumin  appears  in  the 
urine,  hence  retinitis  sufficient  to  attract  the  attention  of  the  patient  is 
indicati\e  of  advanced  degeneration  of  the  kidneys.  It  is  only  in  th? 
early  period  thnt  information  acquired  through  use  of  the  ophthalmosco"  " 
may  be  the  means  of  preserving  life.  In  many  cases  of  contracted  kidney 
albumin  is  not  demonstrable  in  the  urine,  a  fact  which  explains  many 
instances  of  retinitis  reported  as  occurring  in  healthy  subjects. 

History  .\nd  Course.  It  is  difficult  to  estimate  the  frequency  of 
retinitis  in  chronic  nephritis.  From  a  collection  ot  935  cases  of  the  acute 
and  chronic  tvpes.  Parsons  found  the  average  ratio  of  retinitis  to  be  22.4 
'^er  cent.  This  percentage  represents  mostly  cases  of  well  developed 
retinitis,  but  fails  to  include  a  much  larger  number  in  which  minor  vascular 
changes  existed.  Nettleship  collected  43  fatal  cases  in  young  adults,  of 
which  33  had  retinitis. 

As  regards  sex,  nephritic  retinitis  appears  to  be  about  twice  as  fre- 
quent among  men  as  among  women.  Parsons  found  in  180  reported 
cases,  68  per  cent  males  and  32  per  cent  females. 

Retinitis  in  Bright's  disease  occurs  at  all  periods  of  life;  the  usual 
age  is  between  30  and  60  years.  The  voungest  case  reported  is  by  C.  S. 
Bull,  in  a  child  5  years  of  age.  Another,  aged  9  vears  is  reported  by 
de  Schweinitz. 

Retinitis  mav  occur  with  any  form  of  nephritis,  but  is  most  frequent 
in  contracted  kidnev-  It  is  uncommon  with  the  large  white  kidnev  and 
more  rare  in  the  acute  nephritis  of  pregnancy  and  scarlatina. 

The  rourse  of  the  disease  is  eminently  chronic.  Degeneration  of 
the  retina  and  deterioration  of  vision  keep  pace  with  the  advancing  ne- 
phritis. Arrest  or  subsidence  of  the  process  in  chronic  cases  must  be  ex- 
tremely rare,  although  instances  have  been  reported  by  Admuck,  Steffan  and 
others.  Temporary  improvement  in  vision  may  be  due  to  local  causes, 
but  cure  cannot  be  expected  while  the  nephritis  continues.  Uremic  attacks 
may  be  attended  by  temporary  or  permanent  amaurosis. 

The  prognosis  as  to  life  is  extremely  unfavorable  in  fully  developed 
cases  of  albuminuric  retinitis.  In  nearly  all  cases  death  ensues  within  a 
few  months  or  years.  In  103  cases,  C.  S.  Bull  found  that  87  per  cent  died 
within  two  years.     Prognosis  is  no  better  among  the  young  than  the  old. 


Fig.  74. 
Fig.    74 — An.siiopatliic     Albuminuric    Ixetinitis.      R.    retina;    C,    choroid;    S,    sclera. 
Edema  of  the  entire  Retina. 


Fig.  75. 


Fig.  75 — Angiopathic,  Albuminuric  Retinitis.     Cj  stoid  spaces  in  the  internuclear  Layer. 


Fig.  76 — Edematous  Infiltration  around 
ihe  Macula,  in   Albuminuric   Retinitio. 


Fig.  76. 


Fig.  77 — Retinal  Discoloration  left 
after  removal  of  Superficial  Hemorrliage 
by  Leucocytes. 


Fig.  78 — Deep  Hemorrhage  Re- 
placed by  Plaque  of  Cicatricial  Tissue 
with  Pigmented  Borders. 


ANGIOPATIIIC    RMTIXmS.  85 

Nettleship  collc^cted  80  cases  of  chronic  nephritis  in  suhjects  under  21  years 
of  age.  Of  these,  60  came  to  necropsy.  The  influence  of  habit  and  environ- 
ment upon  the  course  of  the  disease  is  forcibly  illustrated  by  the  work  of 
Possauer,  whose  material  was  collected  from  67,000  cases  of  all  kinds. 
In  cases  of  albuminuric  retinitis  among  the  poor  who  visited  the  clinics, 
all  males  died  withi.i  two  years,  a  mortality  of  100  per  cent.  Among 
women  of  the  same  class  only  68  per  cent  died.  Among  private  patients 
of  means,  the  death  rate  was  59  per  cent  for  males  and  53  per  cent  for 
females.  Among  the  poor  tlie  longest  duration  of  life  after  discovery  of 
retinitis  was  6  years,  and  among  private  patients  1 1  years.  These 
statistics  illustrate  the  value  of  good  hygienic  conditions  in  the  treatment 
of  nephritis. 

P.\THOLO(]Y.  The  pathologic  anatomy  of  angiopathic  retinitis  in- 
cludes all  morbid  changes  that  result  from  vascular  disease.  Inasmuch  as 
the  retinal  blood  vessels  arc  a  terminal  system  and  the  only  lymphatics  are 
those  which  accompanvthe  vessels,  any  obstruction  to  circulation  is  followed 
by  disturbance  of  nutrition,  the  visible  manifestations  of  which  are  edema 
and  hemorrhage.  Edema  appears  first  in  the  nerve  fiber  and  inner  re- 
ticular layers,  but  the  entire  retina  soon  is  infiltrated  with  abundant  fluid 
(Fig.  74),  poor  in  albumin  and  readily  absorbed.  The  nerve  fiber  layers 
are  separated  by  the  transudate,  the  inner  layers  being  lifted  up  at  the 
expense  of  the  vitreous  space.  Cystoid  spaces  may  form  in  the  inter- 
nuclear  layer  (Pig.  75).  Edematous  infiltration  is  especially  marked 
around  the  macula  (Fig.  76).  If  edema  is  long  continued  the  nerve  fibers 
swell  and  lose  their  outline.  Transient  stasis  may  not  injure  the  ganglion  cells 
but  these  delicate  structures  are  the  least  resistant  of  the  retinal  elements 
and  quicklv  succumb  in  areas  insufficiently  supplied  with  blood.  Usually, 
the  hemorrhages  are  superficial,  the  blood  Iving  between  the  axons  of  the 
nerve  fiber  laver,  where  they  present  the  ophthalmoscopic  picture  character- 
istic of  striate  or  "  flame-shaped  "  hemorrhages.  Blood  extravasated  into 
the  retina  seldom  clots.  Striate  hemorrhages  are  slowly  removed  by  leuco- 
cytes, leaving  the  retina  slightly  discolored  (Fig.  77).  Deep  hemorrhages 
often  destroy  the  entire  thickness  of  the  retina  which  then  is  replaced  by 
a  plaque  of  cicatricial  tissue  with  pigmented  borders  (Fig.  78).  After 
resorption  of  the  blood  from  the  retina  the  hemoglobin  may  remain  and 
form  pigmented  deposits,  but  the  pigment  around  the  scars  of  deep  retinal 
hemorrhages  is  deposited  by  the  retinal  epithelium.  In  albuminuric  and 
some  other  forms  of  chronic  retinitis,  peculiar  cell-like  bodies,  of  inconstant 
size,  are  found  in  the  ganglion  cell  and  nerve  fiber  layers.     They  are  gran- 


86  THE    FUNDUS    OCULI. 

ular  or  homogeneous  in  structure  and  contain  retractile,  nuclear-like  spots 
(Fig.  79),  the  origin  and  nature  of  which  are  unknown.  They  have  been 
variously  regarded  as  varicose  nerve  fibers,  degenerated  ganglion  or  neu- 
roglia cells  and  as  clusters  of  degenerated  leucocytes.  The  latter  view  is 
at  present  popular.  The  signs  of  active  inflammation  are  wholly  lacking 
in  albuminuric  retinitis,  and  the  white  exudates  so  conspicuous  in  the  oph- 
thalmoscopic picture  are  mostly  the  products  of  degeneration.  They  are 
principally  hyaloid  in  character  but  fat  also  is  present  and,  later,  cho- 
lesterin.  Calcareous  deposits  are  rare.  The  "  star-shape,"  which  exudates 
at  the  macula  often  assume,  corresponds  to  the  radial  arrangement  of 
Henle's  fibers  in  this  region.  The  vascular  changes  are  those  of  arterio- 
sclerosis already  described.  In  the  atrophic  stage  (Stergm.  37)  the  normal 
structures  are  largely  replaced  by  new-formed  fibrous  connective  tissue. 

Ginsberg  has  studied  the  histologic  changes  in  albuminuric  retinitis. 
He  found  that  the  retina  contained  lipoid  substances,  principally  in  the 
sustentaculum.  White  discolorations  of  the  retina  nearly  always  indicated 
subretinal  effusion  and  retinal  exudation.  Vascular  changes  were  com- 
paratively slight,  and  Ginsberg  regards  them  as  unimportant  in  the  pro- 
duction of  albuminuric  retinitis. 

Acute  Albuminuric  Retinitis  (Exudative  Retinitis).  This  form 
of  retinitis  occurs  in  the  albuminuria  of  pregnancy,  scarlatina  and  other 
exanthemata,  and  from  certain  poisons,  especially  lead.  It  Is  best  known 
in  connection  with  pregnancy  and  only  in  this  relation  will  It  be  discussed. 

The  ophthalmoscopic  picture  of  acute  albuminuric  retinitis  Is  the  same 
as  that  presented  by  the  chronic  form  of  retinitis  during  the  exudative 
stage,  but  minus  the  arteriosclerotic  changes  (Stergm.  35).  The  special 
inflammatory  character  of  the  acute  form  upon  which  some  authors  insist, 
is  not  demonstrable  with  either  the  ophthalmoscope  or  microscope.  De- 
tachment of  the  retina  is  said  to  be  relatively  more  frequent  than  In  the 
chronic  type.  Occasionally,  the  acute  form  is  followed  by  a  rapidly  pro- 
gressive, chronic  retinitis.  In  such  cases  chronic  nephritis  may  have  pre- 
ceded pregnancv.  The  clinical  differences  between  chronic  and  acute  reti- 
nitis consist  in  etiology,  development  and  course  rather  than  In  ophthalmo- 
scopic findings.  The  arteriosclerotic  changes  of  chronic  retinitis  are  not 
found  in  the  acute  disease,  but  its  angiopathic  character  is  suggested  by 
bleeding  and.  In  one  case,  by  closure  of  the  central  artery  (Groenouw). 
The  fundus  changes  are  not  necessarily  pronounced.  In  none  of  Nettle- 
ship's  cases  was  the  retinitis  severe. 

Silex  estimates  that  one  case  of  albuminuric  retinitis  occurs  In  every 


ANGIOPATHR-    Kl'.TIMlIS.  8/ 

3,000  pregnancies.  Tn  true  cases,  /.  e.,  those  in  which  chronic  nephritis  does 
not  antedate  pregnancy,  preliminary  vascular  disease  appears  to  be  lacking. 
When  retinitis  occurs  it  usually  does  so  in  the  second  half  of  pregnancy, 
although  it  may  appear  at  any  time.  The  v^iew  that  most  cases  of  retinitis 
occur  in  the  first  pregnancy  is  shown  to  be  erroneous  by  Nettleship  who 
found  but  four  primipara  among  nineteen  cases.  A  woman  in  her  first 
preirnancy  may  suffer  from  nephritis  and  retinitis,  and  escape  both  in  sub- 
sequent pregnancies. 

Prognosis  as  to  life  is  good,  but  in  most  cases  vision  is  permanently 
impaired.  Silex  found  but  three  out  of  thirty-five  cases  that  regained 
normal  sight.  Howe  states  that,  when  retinitis  develops  in  the  sesenth 
month  and  abortion  is  not  induced,  permanent  blindness  may  be  expected. 
Occurring  in  the  eighth  month  less  than  one-half  recover.  If  it  does  not 
appear  until  within  the  two  last  weeks  of  pregnancy,  recovery  is  assured. 
Abortion  may  arrest  the  disease  but  does  not  always  insure  reco\'ery.  and 
progressive  failure  of  \ision  may  continue  after  delivery.  Traces  of  the 
disease  usually  remain  in  the  form  either  ot  white  spots  in  the  retina, 
blanching  of  the  nerxe  or  pigmentation  around  the  macula. 

Why  retinitis  occurs  in  acute  nephritis  has  not  been  explained.  The 
statement  that  it  is  due  to  a  "  uremic  state  "  of  the  blood  is  not  lacking  in 
vagueness.  More  likely,  both  acute  nephritis  and  retinitis  have  a  common 
origin.  Acute  diffuse  nephritis  is  attributed  to  the  presence  of  toxic  sub- 
stances in  the  blood  which,  in  the  process  ot  elimination,  are  brought  into 
intimate  contact  with  the  vascular  and  excretory  cells  of  the  kidneys,  and 
thereby  injure  or  destroy  these  structures.  In  a  similar  manner,  the  same 
poisons  circulating  through  the  retina  may  induce  the  endovascular  and 
parenchymatous  changes  of  acute  retinitis.  The  destructive  effect  of  cir- 
culating poisons  that  act  upon  the  vessels  of  the  kidneys  and  retina  need 
not  be  ascribed  to  "  selective  action,"  but  to  the  anatomic  fact  that  both 
structures  are  supplied  by  a  terminal  system  of  vessels  and  the  evils  of 
obstructed  circulation  cannot  be  averted  by  collaterals.  Nephritis  and  reti- 
nitis may  be  caused  by  either  endogenous  or  exogenous  toxins. 

Uremic  amaurosis  usually  occurs  in  the  course  of  acute  nephritis, 
seldom  Avith  chronic  nephritis.  As  a  rule,  the  funcius  is  normal  or  nearly 
so.  Sometimes,  however,  uremic  amaurosis  develops  in  connection  with 
retinitis,  and  then  it  is  of  vital  importance  to  recognize  the  cause  of  blind- 
ness. A  typical  case  of  uremic  amaurosis  commences  with  rapid  loss  of 
vision  which  in  from  8  to  24  hours  eventuates  in  blindness,  even  light  per- 


88  THE    FUNDUS    OCULI. 

ception,  as  a  rule,  being  abolished.  This  state  continues  for  from  10  to 
18  hours,  when  improvement  ensues  and  after  about  48  hours  vision  is 
rjstored.  In  most  cases  during  the  blind  period  the  pupils,  respond  to 
light.  During  the  attack  the  urine  is  diminished  in  amount,  and  if  albumin- 
uria was  present  it  may  disappear.  When  amaurosis  develops  in  pregnancy 
it  frequently  is  accompanied  by  eclampsia.  In  scarlatina  it  occurs  during 
desquamation.  Uremic  amaurosis  appears  to  be  more  infrequent  than 
albuminuric  retinitis.  Litzman,  in  13  cases  of  uremic  eclampsia  of  preg- 
nancy, found  amaurosis  in  three.  The  unconscious  condition  of  the  patient 
with  eclampsia  often  hides  an  existing  amaurosis.  The  prognosis  as  to 
vision  is  good  in  those  who  survive  the  nephritis.  Recurring  attacks,  how- 
ever, may  result  in  permanent  amblyopia.  Two  principal  theories  exist 
as  to  the  origin  of  the  blindness:  (1)  high  blood  pressure,  which  causes 
edema  of  the  brain;  (2)  the  irritating  action  of  an  unknown  toxin.  The 
persistence  of  pupillary  light  reaction  indicates  a  cerebral  lesion  above  the 
primary  optic  centers,  presumably  cortical. 

Exudative  (Hyperemic:  Congestive  Hemorrhagic)  Stage.  With 
the  dcA'elopment  of  chronic  nephritis,  a  retinitis  is  no  longer  "  precursory." 
The  exudative  stage  of  chronic  retinitis  which  then  ensues  includes  a  wide 
range  of  morbici  conditions,  from  hyperemia  of  nerve  and  retina,  to  a 
state  of  intense  congestion  with  exudation  and  hemorrhage.  With  the 
establishment  of  distinct,  albuminuric  retinitis,  the  disc  becomes  markedly 
congested  and  its  outlines  are  blurred.  Usually,  it  is  moderately  swollen. 
The  retina  becomes  edematous  and  opaque,  especially  around  the  disc, 
where  thickest.  Exudates  appear  first  as  minute,  white,  fluffy  dots  in  and 
around  the  macula.  Later,  yellowish  spots  develop  in  the  swollen  retina 
about  the  nerve.  Small,  striate  hemorrhages  may  occur  among  the  exu- 
dates and  even  on  the  disc.  Visible  v^ascular  changes,  if  present,  may  be 
obscureci  by  the  existing  inflammation.  In  other  cases  the  vessels  may 
exhibit  advanced  degeneraton  from  the  first  for,  although  the  retinitis  is 
young,  the  arteriosclerosis  is  old.  In  the  early  stage  of  retinitis  it  is  not 
uncommon  to  find  "  silver  wire  arteries,"  white  bordered  "vessels  or  other 
angiosclerotic  changes.  In  any  event,  the  veins  are  abnormally  broad  and 
tortuous  and  the  arteries  of  normal  width  or  narrowed.  As  the  disease 
progresses  the  congestive  and  exudative  processes  increase  in  severity. 
The  engorged  and  tortuous  veins  burrow  in  the  swollen  retina.  Deep 
hemorrhages  occur  and  in  some  cases  blood  is  extravasated  into  the  vit- 
reous.     Profuse  bleeding  is  regarded  as  a  grave  prognostic  sign.      The 


ANGIOPATHIC    RETINITIS.  89 

sexere  symptoms  of  this  stage  may  develop  with  such  rapidity  that  the 
incipient  processes  escape  observation.  Sometimes  the  course  of  the  dis- 
ease is  marked  by  acute  exacerbations. 

Stkrkogkam  3S.  Exudative  Stage  of  Angiopathic  Albumin- 
uric Retinitis.  Right  eyeground  ot  a  man  37  years  of  age.  Has  ad- 
vanced chronic  interstitial  nephritis.  One  month  ago  he  first  noticed 
obscuration  of  vision  in  both  eyes.  "Fhe  fundus  of  the  right  eye  presents 
an  unusually  characteristic  picture  of  angiopathic  retinitis  in  the  exu- 
dati\'e  stage.  The  nerve  is  intensely  red,  its  outline  is  obscured  but  swell- 
ing is  not  demonstrable  with  the  ophthalmoscope.  The  arteries  possess  a 
very  bright  central  light  streak  and  their  walls  hide  underlying  veins. 
The  veins  are  somewhat  dilated  and  tortuous  the  tortuosity  being  par- 
ticularly marked  in  the  venous  terminals.  The  disc  is  surrounded  by  a 
number  of  small  striate  hemorrhages.  On  the  temporal  side  of  the  nerve, 
the  retina  contains  numerous  small,  superficially  situated,  Hutiy  exudates, 
some  of  which  cover  the  retinal  \essels.  The  supratemporal  vein  is  sur- 
rounded by  a  large  exudation  in  the  center  of  which  is  a  small  extrava- 
sation of  blood. 

DiA(]NOSis.  That  the  pathologic  process  in  this  fundus  is  an  angio- 
pathic retinitis  is  indicated  by  the  red  nerve,  congested  blood  vessels,  hemor- 
rhages and  superficial  exudates  which  overlie  the  vessels.  The  absence  of 
flat,  sharply  outlined  plaques  of  degeneration  (macular  star,  etc.),  prove 
that  the  retinitis  has  not  as  yet  entered  the  stage  of  degeneration. 

Degenerative  Stage.  This  stage  is  characterized  by  gradual  sub- 
sidence of  retinal  hyperemia  and  edema.  In  course  of  time,  the  thickened 
peripapillary  area  loses  its  reddish-yellow  opacity  and  the  retina  appears 
thin  and,  sometimes,  striped  (wrinkled).  White  spots  of  degeneration 
form  mostly  about  the  nerve,  rarely  as  far  forward  as  the  equator.  These 
spots  are  irregular  in  outline,  free  from  pigment  anci  situated  on  the  sur- 
face of  the  retina  where  they  may  cover  the  vessels.  In  the  exudative 
stage  they  present  a  woolly,  soft-edged  appearance.  The  degenerative 
plaques  appear  flat,  hard  and  sharply  outlined.  Magnus  states  that  large 
plaques  may  be  preceded  by  diffuse  opacity  of  the  retina  which  subsequently 
becomes  white.  Additional  minute  glistening  specks  may  be  scattered  about 
the  posterior  pole.  Frequently,  a  broad  white  zone  forms  around  the 
nerve,  producing  an  appearance  which  has  been  compared  to  a  "  snow- 
bank." Around  the  macula  there  is  an  increase  in  the  number  of  white 
dots  which  sometimes  fuse  and  form  an  encircling  ring,  or  they  may  be 
arranged    in   white    rows   or   lines,    radiating    from   the    fovea    like    rays 


90  THE    FUNDUS    OCULI. 

constituting  the  "  macular  star,"  at  one  time  supposed  to  be  pathogno- 
monic of  albuminuric  retinitis.  The  star  rays  are  unequal  in  length  and 
seldom  form  a  complete  circle. 

In  a  certain  proportion  of  cases,  optic  neuritis  is  developed  sufficiently 
to  justify  the  use  of  the  term  neitroretimtis.  This  combination  may  appear 
at  any  time  previous  to  the  stage  of  atrophy,  after  which  the  nerve  and 
retinal  tissues  become  too  sclerotic  to  manifest  marked  symptoms  of  inflam- 
matory reaction.  In  albuminuric  cases  of  retinal  venous  thrombosis,  neuritis 
is  generally  pronounced  (Stergm.  47).  As  a  rare  event  in  albuminuria, 
optic  neuritis  develops  without  retinitis.  In  such  cases,  after  subsidence 
of  the  swelling,  the  nerve  exhibits  the  white  hue  of  post  neuritic  atrophy. 
The  cause  of  the  neuritis  is  problematical.  Probably,  it  arises  from  arterio- 
sclerotic changes  in  vessels  distributed  to  the  disc  although  some  cases 
may  be  due  to  cerebral  complications. 

In  rare  cases,  vascular  degeneration  in  the  choroid  may  produce  visible 
changes  in  the  fundus,  opacities  in  the  vitreous  or  small  detachments  of 
the  retina,  especially  around  the  nerve.  Extensiv^e  detachment  of  the 
retina,  not  necessarily  dependent  upon  the  choroid,  also  may  occur. 

The  ophthalmoscopic  features  of  the  different  stages  of  albuminuric 
retinitis  may  appear  singly  or  in  the  most  varied  combinations.  Cases 
presenting  all  the  characteristics  of  any  stage  are  rare.  Albuminuric 
retinitis  usually  is  observed  in  the  degenerative  stage  In  which  the  white 
foci  are  the  conspicuous  features,  especially  the  macular  spots  or  star. 

Hemorrhagic  Albuminuric  Retinitis.  This  term  is  applied  to  a 
rare  condition  which  nearly  always  occurs  in  subjects  who  have  advanced 
senile  arteriosclerosis  associated  with  albuminuria.  It  is  characterized  by 
recurrent  disseminated  hemorrhages  in  the  retina,  and  the  absence  of 
neuroretinal  changes  belonging  to  albuminuric  retinitis.  The  fundus  repre- 
sented by  stereogram  32  is  a  good  example,  of  this  condition.  An  analo- 
gous tvpe  of  hemorrhagic  retinitis  occurs  in  diabetes  also  due  to  vascular 
degeneration. 

In  the  retinitis  of  chronic  nephritis,  vascular  degeneration  is  the 
underlying  pathologic  factor.  This  may  cause  closure  of  the  central  artery 
or  central  vein,  producing  effects  which  dominate  the  scene  and  obscure  other 
evidence  of  the  disease. 

Stereogram  36.  Angiopathic  Albuminuric  Neuroretinitis, 
Degenerative  Stage.  Right  eyeground  of  a  man,  42  years  of  age, 
with  advanced  Interstitial  nephritis.  The  retina  is  passing  from  the  exu- 
dative to  the  degenerative  stage  of  retinitis.     The  case  is  complicated  by 


ANGIOPATHIC    RKTINITIS. 


91 


a  rather  se\cre  neuritis  which  serves  to  increase  the  tortuosity  of  the  veins 
and  retinal  edema.  The  diiierent  steps  of  albuminuric  retinitis  which  have 
been  described  are  never  sharply  differentiated.  For  example,  in  this 
fundus  the  retinal  edema,  hemorrhages  and  fluffy  exudates  belong  to  the 
stage  of  exudation  or  congestion,  but  the  stellate  figure  about  the  macula 
ancl  the  large  whitish  patches,  sul^ce  to  place  the  case  in  the  degenerative 
stage.  1  he  retinal  edema  is  most  conspicuous  about  the  nerve.  The 
outlines  of  the  disc  are  completely  obscured  and  the  nerve  head  appears 
as  a  muddy,  red  blur.  The  engorged  and  tortuous  veins  burrow  into  the 
swollen  retina  and  disappear.  The  arteries  are  narrowed  and  their 
central  light  streaks  are  sharp,  bright  and  irregular.  The  hemorrhages 
are  striate,  indicating  that  they  lie  in  the  nerve-fiber  layer.  The  large 
whitish  plaques  are  superficially  situated,  well  defined  and  nonpigmented. 
The  spots  cover  the  arteries  when  the  two  are  in  contact.  The  "  star  "  does 
not  completely  encircle  the  macula.  Its  rays  consist  of  short  linear  and 
rounded  whitish  spots  arranged  in  rows.  Small,  fluffy,  edematous  spots 
are  seen  where  two  vessels  cross.  A  group  of  small  dots  is  scattered 
among  the  superior  temporal  vessels. 

Di.\(]\()Sis.  In  the  great  majority  of  cases  angiopathic  retinitis  occurs 
in  connection  with  chronic  nephritis,  l^xceptionallv,  it  de\'elops  in  other 
affections  attended  by  vascular  degeneration.  Occurring  iii  diabetes  the 
ophthalmoscopic  picture  may  or  may  not  present  characteristic  features. 
In  diabetic  retinitis,  visible  angiosclerosis,  optic  neuritis  and  opacity  of  the 
retina  are  comparatively  rare.  It  must  be  borne  in  mind  that  unrecognized 
nephritis  may  exist  In  connection  with  any  disease  which  exhibits  angiopathic 
retinitis,  particularly  diabetes.  Albuminuric  retinitis  in  the  degenerative 
stage  requires  differentiation  chieflv  from  chronic  choroiditis. 


Al-Rl^MIXURIC     Rf.TIXITIS. 


Occurs  chieflv  in  posterior  polar 
zone. 

White  patches,  superficlallv  situ- 
ated; o\erlIe  retinal  vessels; 
edges    never   pigmented. 

Veins  dilated.  Arteries  normal 
width  or  narrowed.  Visible 
angiosclerosis. 


C  [IRQ  NIC    CnoRoiniTis. 

Lesions      distributed      throughout 
fundus. 

White    patches,     deeply    situated; 

always   beneath    retinal    vessels; 

old     lesions     looked     "  punched 

out";     more     or     less     pigment 

disturbance. 
Retinal  vessels  normal  or  uniformly 

narrowed  in  old  cases. 


92 


THE    FUNDUS    OCULI. 


ALBUMINURIC    RETINITIS. 

Choroidal   vessels   not   exposed. 
More  or  less  optic  neuritis. 
Retinal  hemorrhages. 
Mtreous  opacities  are  unusual. 


Chronic  Choroiditis. 


Some  choroidal  vessels  usually  ex- 
posed. 

Neuritis  is  very  rare. 

Hemorrhage  is  exceptional. 

\"itreoiis  opacities  common  in  early 
stage. 


Albuminuric  retinitis  is  differentiated  from  other  angiopathic  forms 
by  diagnosticating  the  associated  general  disease. 

Atrophic  Stage.  Retinitic  Optic  Atrophy.  In  natural  se:]uence, 
the  stage  of  degeneration  is  followed  by  that  of  atrophy.  All  signs  of 
retinal  opacity,  hyperemia  and  neuritis  subside  and  the  red  fundus  reflex 
reappears.  As  in  other  forms  of  retinal  atrophy,  the  fundus  remains 
delicately  veiled.  The  white  plaques  of  the  degenerative  stage  become 
indistinct,  changing  from  white  to  a  dirty  grey  and  finally  disappear  or 
remain  as  patches  of  scattered  grey  specks.  1  he  last  to  disappear  is  the 
stellate  figure  around  the  macula.  Degeneration  of  the  retina  leads  to 
retinitic  optic  atrophy,  characterized  by  the  yellowish-white  color  of  the 
disc.  If  severe  neuritis  existed  in  the  earlier  stages,  the  disc  may  now 
present  the  white  color  and  peripapillary  pigment  disturbance  of  post- 
neuritic atrophy.  Scattered  pigment  deposits  may  appear  either  ircm 
choroidal  degeneration  or  from  extravasated  blood.  The  blood  vessels 
undergo  extreme  degeneration  and  the  smaller  branches  disappear.  1  his 
ophthalmoscopic  picture  is  very  r:ire  for  the  reason  rhat  albuminuric 
subjects  seldom  live  long  enough  for  the  retinitis  to  reach  this  stage. 

Stereogram  37.  Angiopathic  Albuminuric  Retinitis.  Atro- 
phic Stage.  Teft  eyeground  of  a  woman  29  years  of  age.  Urinary  ex- 
amination indicates  advanced  cirrhosis  of  the  kidneys.  Three  months 
ago,  in  the  3e\'enth  month  of  her  third  pregnancy,  she  became  bli-id  from 
retinitis.  An  abortion  was  induced  and  vision  was  partly  restored. 
Her  vision  was  defective  before  the  last  pregnancy.  Ophthalmoscopic  ex- 
amination revealed,  in  both  eyes,  angiopathic  retinitis  in  the  atronhic 
stage.  Considering  the  advanced  stage  of  retinitis  that  now  exists,  it  is 
probable  that  pregnancy  was  an  intercurrent  event  in  the  course  of  a 
chronic  nephritis.  On  the  other  hand,  chronic  nephritis  with  retinitis, 
following  an  acute  attack,  sometimes  pursues  a  remarkably  rarid  course. 

The  optic  nerve  presents  the  yellowish-white  color  of  retinitic  atrorhy. 
The  fundus,  especially  on  the  nasal  side,  contains  several  dirty-grey  patches 


AXGIOPATHIC    RETIXniS.  93 

which  are  interpreted  as  retinal  cicatrices  that  have  replaced  the  white 
plaques  of  the  dei^enerative  stage.  In  accordance  with  the  rule,  the  last 
spots  of  exudate  to  disappear  are  those  around  the  macula.  In  this  case, 
the  few  which  remain  may  be  remnants  of  a  macular  star.  Just  above 
the  macular  region  are  three  small,  shining,  white  specks,  probably  crystals 
of  cholesterine.  Several  masses  of  pigment  are  grouped  along  the  course 
of  a  macular  vein.  These  are  regarded  as  of  hematogenous  origin  for 
the  following  reasons:  (1)  they  are  superficially  situated  upon  dirty- 
vellow  patches,  such  as  remain  after  resorption  of  blood;  (2)  the  choroid 
is  not  ex])osed;  (3)  pigment  is  within,  not  around  the  patch;  (4)  evident 
relationship  of  the  jiigmcnt  to  a  blood  vessel.  \'aried  and  irregular  vas- 
cular degeneration  is  characteristic  of  primary  angiosclerosis  as  distin- 
guished from  the  uniform  narrowing  and  obliteration  of  vessels  due  to 
retinal  atrophy  (Stergm.  44).  All  the  blood  columns  are  either  irregu- 
larlv  constricted  or  widened.  I  he  superior  temporal  arteries  exhibit 
sectional  sclerosis.  These  vessels  form  a  series  ol  fusiform,  thin  walled 
dilations  alternating  with  stretches  of  arterv  in  which  the  wall  is  thickened 
and  lumen  constricted.  The  thickened  walls  of  the  constricted  sections 
are  not  visible.  It  is  said  that  such  translucent  thickening  is  due  to  pro- 
liferation of  the  intimi  (endarteritis  nodosa).  The  ascending  branch  of 
this  artery  is  faintly  N'isible  as  it  passes  beneath  a  vein.  This  is  evidence 
of  thickened  arterial  walls.  1  he  supranasal  arterv  also  exhibits  sectional 
thickening  and  its  walls  are  visible  as  white  lines  bordering  the  narrowed 
blood  column.  When  vessel  walls  become  \isible  it  is  supposed  that  the 
sclerosing  process  has  extended  to  the  adventitia.  At  its  bifurcation,  each 
branch  of  this  artery  undergoes  dilation.  Both  the  ascending  and  descend- 
ing retinal  arteries  hide  underlying  veins  on  the  disc,  and  the  spaces  which 
here  appear  to  exist  between  the  ends  of  the  veins  and  arterial  blood 
columns,  measure  the  thickness  of  the  arterial  walls.  The  veins  present 
irregular  blood  columns.  The  smaller  veins  and  terminals  are  tortuous. 
Both  \  enous  and  arterial  twigs  have  been  extensively  obliterated  by  the 
general  retinal  atrophy.  On  the  disc,  the  sclerosed  arteries  press  on  the 
underlying  veins  and  obstruct  the  venous  flow.  This  is  manifested  by  local 
dilation  of  the  veins  distal  to  the  points  of  pressure. 

The  thin  patches  of  atrophic  retinitis  could  hardly  be  mistaken  for 
the  deep,  pigmented  lesions  of  choroiditis  (Stergm.  20).  Other  forms  of 
retinal  atrophy,  i.  e.,  retinitis  pigmentosa  (Stergm..  S3),  puncata  albescens 
(Stergm.  54),  occlusion  atrophy  (Stergm.  44),  etc.,  all  possess  char- 
acteristic  fundus   changes.      Moreover,    in   all   these   diseases   the    retinal 


94  THE    FUNDUS    OCULI. 

vessels  undergo  uniform  narrowing  and  obliteration  while  in  atrophy 
following  angiopathic  retinitis  the  vessels  present  the  varied  degenerative 
changes  of  arteriosclerosis. 

i\NGlOPATHic  DiABETic  RETINITIS.  The  retinitis  which  occurs  in 
saccharine  diabetes  presents  no  ophthalmoscopic  feature  by  which  it  can 
with  certaintv  always  be  differentiateci  from  other  forms  of  angiopathic 
retinitis.  Peculiarities,  however,  exist  which,  in  typical  cases,  permit  a 
probable  diagnosis.  Thus,  the  visible  angiosclerotic  changes  so  prominent 
in  albuminuria  are  usually,  but  not  invariably,  absent  in  the  retinitis  of 
diabetes.*  Optic  neuritis  and  opacity  of  the  retina  also  are  very  rare. 
The  frequencv  of  both  albumin  and  sugar  in  the  urine  has  rendered  it 
difficult  to  Isolate  a  special  diabetic  type  of  retinitis  which  is  free  from 
suspicion  of  being  a  case  of  albuminuric  retinitis  in  a  diabetic  subject. 
Retinitis,  however,  does  occur  in  diabetes  in  the  absence  of  albuminuria. 
HIrschberg  has  separated  uncomplicated  cases  into  two  classes  which 
may  be  accepted  as  typical  of  saccharine  diabetes.  These  are:  (1)  Cen- 
tral punctate  diabetic  retinitis;  (2)  Hemorrhagic  diabetic  retinitis. 

Central  Punctate  Diabetic  Retinitis.  In  this  type  there  is 
Utile  or  no  evidence  of  vascular  disease,  optic  neuritis  or  retinal  edema. 
In  the  region  embraceci  between  the  superior  and  Inlerior  temporal  vessels 
are  groups  of  white  spots  and  lines.  Ihey  are  grouped  chiefly  around  the 
macula,  but  do  not  form  a  star.  Smaller  collections  occur  between  the 
macula  and  nerve.  Isolated  spots  may  appear  outside  the  temporal  area, 
or  on  the  nasal  side  of  the  disc,  or  in  front  of  the  vessels.  They  exhibit 
no  tendency  to  coalesce  and  large  spots  are  uncommon.  1  he  spots  and 
stripes  are  Irregular  in  outline  and  their  edges  may  be  serrated.  Blood 
is  seldom  seen  on  the  spots,  but  ev^erywhere  between  them  are  small,  super- 
ficial hemorrhages.  Hemorrhages  occur  also  beyond  the  area  of  the 
spots.     Pigment  changes  are  lacking  and  the  vitreous  Is  clear. 

Stereogram  38.  Central  Punctate  Diabeiic  Retinitis.  Left 
eyeground  of  a  laborer,  52  years  of  age.  His  vision  first  began  to  fail 
seven  months  ago  and  now  is  20/200  in  each  eye.  The  urine  contains 
sugar,  3  per  cent;  no  albumin,  no  casts  and  no  acetone.  Examination  of 
the  feces  shows  marked  pancreatic  Insufficiency.  The  fundus  changes  are 
symmetrical  in  each  eye.  The  ophthalmoscopic  picture  is  a  remarkable 
example  of  Hirschberg's  central,  punctate  diabetic  retinitis.  The  optic 
disc   is   normal    and   the    retinal   vessels   are   not   visibly   sclerosed.      The 


*Cases     of    visible     arteriosclerotic     change.-     in     diabetic     retiniti>,     reported     by 
Galezowski,    Michel,    Knapp. 


ANGiopA'rmc   KKTixins.  95 

region  embraced  by  the  upjier  and  lower  temporal  \essels  is  covered  by 
groups  of  superficially  situated,  small,  white,  discrete,  sharply  defined  spots, 
many  of  which  possess  an  elongated  shape.  Scattered  between  the  spots  are 
numerous  small  striate  hemorrhages.  A  few  isolated  spots  are  situated  o  i 
the  nasal  side  of  the  nerve. 

The  patient  was  kept  in  the  hospital  ward  for  several  weeks,  during 
which  time  no  improvement  occurred. 

l.^iAGNOSis.  This  picture,  which  is  extremely  rare,  probably  is  char- 
acteristic of  diabetes,  although  the  several  types  of  angiopathic  retinitis 
are  differentiated  by  physical  examination  rather  than  by  the  ophthalmo- 
scope. The  angiopathic  character  of  this  retinitis  is  indicated  by  the  hemor- 
rhages. It  differs  in  appearance  from  albuminuric  retinitis  in  the  absence 
of  neuritis,   visible  vascular  changes  and   retinal   edema. 

HivMOKRiiAGic  DiABKTir  Rktixitis.  This  condition  appears  to  be 
analogous  to  the  hemorrhagic  retinitis  of  albuminuria.  It  is  distinguished 
by  the  repeated  occurrence  of , retinal  hemorrhage  and  by  the  absence  of 
either  white  spots  or  visible  vascular  degeneration.  Any  form  of  retinal 
hemorrhage  mav  occur.  When  disseminated,  the  extravasations  are  said 
to  be  rounder  and  more  deeply  situated  in  the  retina  than  In  the  al'-iumlnurlc 
type.  Small  punctlform  hemorrhages  in  the  retina  are  suggestive  of 
diabetes.  Intravitrcous  bleeding  and  vitreous  opacities  are  frequent  in 
this  type  of  diabetic  retinitis.  Posthemorrhagic  glaucoma  may  develop  and 
invariably  is  destructive. 

Under  the  name  of  atypical  d'lahclic  retinitis  Hirschberg  places  a  third 
group  of  cases  in  which  diabetes  is  accompanied  by  tvplcal  retinitis  pig- 
mentosa, but  a  causal  relationship  between  the  two  conditions  has  not  been 
demonstrated. 

Diabetic  retinitis  Is  a  rare  disease,  only  a  few  observers  having  seen 
a  sufficient  number  of  cases  to  be  convinced  that  it  possesses  any  special 
characteristic  feature.  The  case  of  central  punctate  retinitis  described 
above,  so  closely  corresponds  to  Hirschberg's  cases,  that  I  am  inclined  to 
regard  the  picture  as  nearly  pathognomonic  of  diabetes.  A  large  pro- 
portion of  reported  cases,  however,  were  ophthalmoscopically  indistin- 
guishable from  albuminuric  retinitis.  Even  the  macular  star  Is  not  un- 
known in  diabetes,  five  cases  having  been  reported,  three  by  Hirschberg, 
one  by  Hirschmann  and  one  by  Hawthorne. 

Although  the  walls  of  the  blood  vessels  are  seldom  opaque,  diabetic 


96  THE    FUNDUS    OCULI. 

cases  are  not  exempt  from  profound  vascular  ciisease,  as  shown  not  only 
by  hemorrhage,  but  by  cases  of  thrombosis  of  the  central  vein*  and 
closure  of  the  central  artery. t 

tipemia  is  an  occasional  complication  of  diabetes.  In  such  cases  the 
retinal  vessels  appear  filled  with  a  milky  fluid.  The  arteries  have  a  reddish 
and  the  veins  a  violet  tint.  Uthoff  thinks  this  appearance  is  produced  by 
the  fat  globules  which  float  on  the  surface  of  the  blood  column,  forming 
a  layer  in  contact  with  the  walls  of  the  vessels. 

Functional  disturbance  of  vision  is  far  greater  in  diabetes  than  in 
albuminuria.  '\' arious  types  of  amblyopia  occur,  with  and  without  retinitis. 
Amblyopia  with  sector  defects  m  the  field  is  common.  Optic  atrophy  with 
concentric  contraction  has  been  reported.  Schobl  states  that  diabetics 
frequentlv  complain  of  "  glimmering  "  followed  by  central  amblyopia. 
Affection  of  the  maculo-papillary  bundle,  indistinguishable  from  nicotine 
amblyopia  may  develop.  Mauthner  justly  regards  these  cases  as  due  to 
the  use  of  tobacco  and  alcohol,  as  diabetic  subjects  are  very  susceptible  to 
the  action  of  these  and  other  poisons.  Aside  from  retinal  anci  nerve  af- 
fections, vision  in  diabetics  is  prone  to  be  disturbed  by  imbalance  of  the 
extrinsic  muscles  and  also  by  paresis  of  accommodation  and  premature  pres- 
byopia.    C  ataract  is  a  frequent  event  in  diabetes. 

Retinitis  is  a  late  development  of  diabetes  and  is  to  be  regarded  as 
an  unfavorable  symptom,  although  the  prognosis  as  regards  life  is  far 
better  than  in  abuminuria.  After  the  development  of  diabetic  retinitis 
the  general  and  local  conditions  often  remain  unaltered  for  a  long  period. 
Nettleship,  in  48  cases,  found  that  60  per  cent  lived  more  than  two  years. 

Angiopathic  Leukemic  Retinitis.  Leukemia  is  manifested  in 
the  retina  either  by  neuroretinitis  or  by  retinal  hemorrhages  without 
other  inflammatory  phenomena. 

Leukemic  Neuroretinitis.  In  this  form  the  hyperemia  usually  is 
of  moderate  intensity.  In  some  but  not  all  cases,  the  disease  is  distinguished 
by  a  pale,  orange-yellow  color  of  the  fundus  and  by  white  centered  hemor- 
rhages. Sometimes  the  blood  vessels  may  be  nearly  white  in  color  the 
v^eins  being  distinguished  only  by  their  excessive  size  and  tortuosity.  In 
other  cases  the  vessels,  especially  the  veins,  exhibit  white  borders.  Retinal 
opacity  is  present,  but  seldom  so  pronounced  as  in  albuminuric  retinitis. 
Escape  of  blood  into  the   retina  is  one  of  the  most  common  events   in 


*V.   Michel;    Hirsrhberj 
tKnapp;    Dodd. 


ANGIOPATHIC    RETINITIS.  97 

leukemia.  The  extravasated  blood  is  said  to  be  lighter  in  color  than  in 
other  diseases.  Fresh  extravasations  often  present  a  characteristic  ap- 
pearance, consisting  of  a  central  white  spot  surrounded  bv  blood.  Gen- 
erally the  hemorrhages  are  situated  in  the  nerve  fiber  laver  or  on  the 
surface  of  the  retina.  If  bleeding  is  profuse  all  layers  of  the  retina  or 
even  the  \  itreous  may  be  invaded.  1  he  lesions  of  leukemic  retinitis  fre- 
quently are  limited  to  the  region  anterior  to  the  equator  and  thus  escape 
obser\aii()n.  The  neuritis  varies  greatly  in  different  cases.  Although 
usuallv  moderate  in  degree,  it  may  be  excessive,  the  nerve  projecting  like  a 
mushroom  into  the  vitreous.  Occasionally  the  leukemic  fundus  presents 
the  picture  of  "  albuminuric  retinitis."*  Michel  has  described  two  cases  of 
retinal  venous  thrombosis  in  leukemia. 

The  pathologic  changes  of  leukemic  retinitis  are  due  to  the  excess 
of  leucocvtes  in  the  blood  ard  their  accumulation  in  the  tissues.  The 
orange  color  of  the  fundus  is  supposed  to  be  caused  by  lymphatic  infil- 
tration of  the  choroid.  Fhe  perivascular  sheaths  may  be  filled  with  lymph 
cells  and  appear  as  white  lines  bordering  the  retinal  vessels  or  may  even 
whiten  the  entire  vessel.  Similarly,  choked  disc  may  result  from  infil- 
tration of  the  nerve  and  nerve  sheaths  with  leucocytes.  The  bleeding  in 
leukemia  is  explained  by  the  altered  state  of  the  blood.  The  great  excess 
of  the  white  cells  permits  them  to  accumulate  along  the  walls  of  the  vessels, 
through  which  they  pass  with  the  red  cells.  Michel  attributes  the 
bleeding  to  venous  thrombi.  Probably,  Saemisch  is  correct  in  regarding 
the  white  centers  of  the  hemorrhages  as  composed  of  white  blood  cells. 
Leber's  suggestion  that  they  are  small  lymphomata  has  not  been  confirmed. 
Leukemic  extravasations  possess  the  following  peculiarity.  In  the  retinal 
hemorrhages  of  other  diseases,  white  areas  form  only  in  old  extrava- 
sations and  represent  the  processes  of  blood  resorption  and  degeneration; 
while  the  white  center  of  a  leukemic  hemorrhage  appears  when  the  blood 
is  extravasated.  Examples  of  both  tvpes  ot  white  spots  are  found  in 
leukemic  retinitis.  Microscopic  examinations  have  thrown  little  light  on 
the  stri!Cture  of  leukemic  extravasations,  inasmuch  as  only  old  hemor- 
rhages hnve  been  described. 

Deterioration  of.  vision  in  leukemia  appears  to  depend  upon  actual 
lesions  in  the  re.ina  and  ner\-e  rather  th'-n  on  amblyopias  of  toxic  or  cerebral 
origin.  Schmitt-Rlmpler,  however,  has  reported  a  case  associated  with 
retrobulbar  neuritis. 


*Schirmer;     Kramsztky. 
7 


98  THE    FUNDUS    OCULI. 

Other  ocular  manifestations  of  leukemia  are  lymphoma  of  the  eyelids, 
exophthalmus  due  to  lymphogenous  deposits  or  to  hemorrhage  in  the 
orbit,  enlargement  of  the  lachrymal  gland,  iritis,  iridocyclitis  and  hyphema. 

Hemorrhagic  Leukemic  Retinitis.  In  this  form  of  retinitis  the 
signs  of  congestion  are  slight  or  lacking.  The  fundus  may  or  may  not 
present  the  yellowish  cast  of  leukemia.  Some  dilation  of  the  v^eins  usually 
exists.  In  the  following  case  the  most  interesting  clinical  feature  was  the 
presence  of  a  white  center  in  a  fresh  hemorrhage. 

Stereogram  39.  Leukemic  Memorrhagic  Retinitis.  Right  fun- 
dus oculi  of  a  man  32  years  of  age.  Syrian.  Entered  the  clinic  complaining 
only  of  slight  conjunctivitis.  He  was  intensely  anemic.  Blood  examination 
demonstrated  the  presence  of  lymphatic  leukemia.  Urine  normal.  Vision 
20/20.  With  the  ophthalmoscope,  numerous  characteristic  white  centered 
hemorrhages  along  the  arteries  and  veins  were  found  in  both  eyes.  Three 
days  after  coming  under  observation,  central  vision  in  the  right  eye  was 
suddenly  lost  during  a  fit  of  sneezing.  I  saw  him  five  hours  after  this  ac- 
cident and  found  a  large,  fresh  hemorrhage  covering  the  macula.  This 
po-y  extravasation  contained  a  round,  sharply  defined,  brilliantly-white 
center. 

The  stereogram  presents  the  ophthalmoscopic  picture  seen  at  this 
time.  Although  the  patient  has  a  very  dark  complexion,  the  fundus  pos- 
sesses a  light,  yellowish  cast  and  the  surface  appears  granular.  This 
appearance  is  attribut^^d  to  an  orange  colored  choroid  shining  through 
deeply  pigmented  retinal  epithelium.  The  optic  disc  has  a  uniform  pink 
color,  but  there  is  no  evidence  of  neuritis.  The  axial  light  streak  on  the 
arteries  is  unduly  broad  and  bright.  The  veins  are  rather  full.  The 
entire  fundus  contains  numerous  striate  hemorrhages  several  of  which  con- 
tain a  round,  white,  central  spot.  Immediately  below  an  artery,  on  the 
nasal  side,  is  a  large  preretinal  hemorrhage.  Beneath  the  supranasal 
vessels  is  a  yellowish  patch,  about  the  area  of  the  optic  disc,  which  marks 
the  site  of  an  old  retinal  hemorrhage. 

The  patient  returned  to  Svria  where  he  died  Irom  leukemia  about 
one  year  later. 

Retinitis  iv  Pernicious  Anemia.  The  retinitis  of  pernicious  anemia 
is  largely  hemorrhagic  In  type.  The  ophthalmoscopic  appearances  resemble 
1;hose  of  hemorrhagic  leukemic  retinitis,  although  Schobl  sees  certain 
peculiarities  in  that  of  pernicious  anemia,  /'.  e.,  a  greyish  cast  to  the  fundus, 
dark  veins  and  nearly  empty  arteries.  Miiller  declares  that  retinal  hemor- 
rhage occurs  in  every  case  of  pernicious  anemia  w^hen  the  disease  is  at  its 


ANGIOPATHIC    RETINITIS.  99 

height.  Certainly,  it  usually  has  been  found  when  sought.  This  point  is 
of  diagnostic  importance  as  retinal  bleeding  is  very  rare  in  chlorosis  and 
simple  anemia.  The  hemorrhages  vary  in  size  and  occur  in  all  layers  of 
the  retina.  White  areas  appear,  said  to  be  identical  with  those  found  in 
leukemia,  but  in  reported  cases  no  distinction  is  made  between  the  spots 
in  new  and  old  extravasations.  Microscopic  examinations  have  shown 
that  the  spots  in  pernicious  anemia  contain  lymphocytes,  phagocytes  and 
cytoid  bodies  (ganglionic  nerve  fibers).  Apparently  these  spots  vary 
little  in  structure  from  those  found  in  any  old  retinal  hemorrhage.  The 
extravasations  are  said  to  be  especially  frequent  around  the  nerve.*  Ret- 
inal edema,  white  and  yellow  spots  in  the  retina  and  a  star  figure  at  the 
macula  have  all  been  seen  in  pernicious  anemia. t  Choked  disc  is  not  an 
unusual  complication.  Many  authors  have  mentioned  the  preseace  of 
\ascu1ar  degeneration  in  this  disease  and  both  Moses  and  Michel  have 
met  thrombosis  of  the  central  \'ein. 

Bihriography. 

Amman,  Beitr    z.  p.  A.  XXXVIII,  p.  1. 

Bettmann,   A.   f.   A..   XI,   1,   p.  2:!. 

Bull.   C.   S.   Am.  J.   Med.   Sci.,   1879;    Trans.   .\ni.   Oph.   Soc.   1S8(>. 

De  Schweinitz,  in  Tyson's  "  Brights  and  Diabetes,"  Trans.  Am.  Oph.  Soc,  1896,   64.5. 

Dodd,  A.  f.  A..  XXXI,  1895. 

Galezowski.   Kecueil   d'O.   187,1.   p.   90. 

Ginsberg,  Arch.  f.  Oph.   LXXXII,   1. 

Greenow,  Parsons   Path,  of  The   Eye,   IV,  p.   1297. 

Hansen,   Nord.   Med.   Ark.,  XII.   1,' 1880. 

Hawthorne,    Lancet,   Sept.   30,    1899. 

Hirschbersf,   C.  f.  p.  A..  X,  1S86;    XV,  1891,  p.  IS. 

Hirschmann,    Inaug.   Dissert.,    Berlin,   1886. 

Howe,   Am.    T.   of   Oph.,   2,   1885,   p.   116. 

Immermann,   Deutsch.   Zeitschf.   f.   Klin.   Med.   XIII,   p.  209. 

Knapp,   A.   f.   A.,  X,   1.   1880,  p.   99 

Kramszky,  Jahresben'cht  f.   Oph.   1878,  226. 

Leber,   Graefe  u.   Saemisch   Handb.   Bd.   5.     Zeits.   f.   Med.   Bliitter.    Bd.   VII,   1869. 

Litzmann,   Deutsch.    KHnik,   1852. 

Ma.gnus,   Rel'.   in   Norris   &   Oliver,   III,   p.   517. 

Mauthner,  Wein  Med.  Klin.  Rundschau,  1893. 

Michel.   Deutsch    Arch.   f.    Klin.   Med.,   XXII,   5   u.   6,   439;    2   Auf.    Lehrb. 

Moses,   Inaug.   Dissert.   Wurtzburg,  1896. 

Miiller,  Die.  Prog.  Pern.  Aneniie,  Zurich,  1877;    Ref.  in   Taherb.  f.  O.  1877,  207. 

Nettleship,   O.   H.  Rep,  XV,  4.   1903;    XVI,   1,   1904;    XT,   1886. 

Parsons,   T.    O.    Soc,   XXIX,    1909,   p.    160;    XXVII,    1907,    p.    119-120;    Path,    of    the 

Eye,  p.  1294. 
Possauer,   Inaug.   Dissert.   Zurich,   1894. 

Quincke,    Deutsch.   A.   f.    Klin.   Med.,   XX,   1;    Volkmann's   Samm.    1876. 
Roth-Schirmer,  A.  f.  Path.  Anat.  XL.  p.  441. 


*Uthofif;  Amman. 

timmermann  ;  Bettman ;  De  Schweinitz;  Sgrosso ;  Miiller;  Hausen  ;  Quincke. 


100  THE    FUNDUS    OCLXI. 

Saeniisch,  Zeitsch.   f.   Med.   Blatter,   Bd.  VII,  1869. 

SchobI,    Nonis   and    Oliver,    III,    pp.    505-513-.522. 

Schlesinger,   Inaus'.    Dissert.   Berlin.   1884;     Ref.   in    Norris   &  O.,   III.   p.   523. 

Sgrosso,   ref.  in   Jahresber.   f.   Oph.   1898,  p    495. 

Silex,    Berlin    Klin.   Woch.   1895,   p.  385. 

Simon,    C.   f    p.   A.,    1894,   p.    132. 

Stcffan,    Jahreshericht    f.    Oi^h.    1873,   p.   348. 

Uthoff,   Intern.   Med.   Cong.,  Paris,  1900;   Klin.  m.  f.  Aug.  1880,  p.  513. 

Wilbrand   u.   .Saenger,   Path.   d.   Netzhaut,   p.   316. 

Angiopathic  Syphilitic  Ritinitis.  In  the  eye  syphiKs  exhibits  a 
preference  for  the  uveal  tract  and  when  it  invades  the  fundi:s  it  usually 
appears  as  a  chorioretinitis  (Stergms.  19-20-21).  Exceptionally,  the  brunt 
of  the  disease  is  borne  by  the  retina,  and  in  rare  cases  only  the  retinal 
vessels  are  affected.  Syphilitic  disease  limited  to  the  inner  retinal  layers 
was  first  described  by  Jacobson  and  later  by  Mauthner.  The  existence  of 
such  a  condition  has  long  been  contesteci.  SchobI  described  five  cases  which 
he  considers  typical  cf  the  condition.  The  symptoms  were:  dusty  opacities 
in  the  posterior  part  of  the  vitreous;  red  blurred  disc;  slight  retinal  edema; 
arteries  narrowed;  veins  dilated;  retinal  hemorrhages.  The  presence  of 
vitreous  opacities  in  Schobl's  cases  would  indicate  that  the  choroid  as  well 
as  the  retina  was  affected.  A  review  of  the  literature  demonstrates  that 
most  cases  present  the  retinal  changes  incident  to  degeneration  of  its  v^essels, 
differing  in  no  w^ay  from  angiopathic  retinitis  as  it  occurs  in  other  diseases. 
A  wall-like  opacity  about  the  nerve  similar  to  that  which  occurs  in  albumin- 
uria, was  found  in  syphilitic  retinitis  by  Classen,  Schweigger  and  Alexander. 
Groups  of  minute,  yellowish  dots,  clustering  about  the  blood  vessels  were 
described  by  Hirschberg.  An  ophthalmoscopic  picture  identical  with  that 
seen  in  albuminuric  retinitis  has  been  observed  in  syphilitics  whose  urine 
contained  neither  albumin  nor  sugar,  by  Knapp,  Flaab  and  Schaffels.  Ar- 
teriosclerotic alteration  in  the  retinal  anti  choroidal  (Fig.  80)  vessels 
without  marked  changes  in  the  retina  is  a  matter  of  common  observation 
in  cases  of  old  syphilis.  Haab  regards  white  scales  on  the  retinal  vessels 
as  characteristic  of  syphilitic  vasculitis.  Cerebral  syphilis  frequently  is 
accompanied  by  sclerosis  of  retinal  vessels,  v.  Hosslin  describes  five  cases 
of  brain  syphilis  which  exhibited  endarteritis  of  the  retinal  vessels,  and  in 
one  of  the  cases  there  was  optic  neuritis.  The  term  "  hemorrhagic  syph- 
ilitic retinitis  "  has  been  applied  to  cases  in  which  syphilitic  vasculitis  re- 
sulted in  extensive  retinal  hemorrhages.  Closure  of  the  central  vessels  and 
their  branches  occurs  in  syphilis  as  in  the  vascular  degeneration  of  other 
diseases.  Closure  of  the  central  artery  has  been  described  by  Stolting  and 
by  Oglesby,  and  Haab  regards  syphilitic  endarteritis  as  responsible  for  many 


ANGIOP.ATHIC    RKTINITIS.  101 

cases.  Glaucoma  developing  in  syphilitics  has  been  attributed  to  specific 
vascular  degeneration.*  Before  accepting  this  conclusion  it  is  necessary  to 
demonstrate  that  glaucoma  can  be  directly  produced  by  disease  of  the 
blood  vesseh. 

Visual  (iisturbance  in  sypliilitic  retinitis  may  be  out  of  proportion  to 
the  visible  changes.  (jlimmering  is  an  early  and  prominent  symptom 
which  Hirschberg  attributes  to  retinal  anemia  caused  by  arterial  disease. 
It  is,  however,  more  reasonable  to  suppose  that  ''  glimmering  "  is  caused 
in  these,  ns  in  other  cases,  by  choroidal  disease.  The  same  explanation 
applies  to  night  blindness,  which  is  a  frequent  symptom  of  syphilitic  reti- 
nitis. Ring  scotomata  and  other  forms  may  develop.  In  syphilitics  with 
angiopathic  retinitis,  the  urine  may  contain  albumin  or  sugar.  In  such  cases 
the  retinitis  should  not  be  classed  as  strictly  syphilitic. 

B'lbriogrupliy. 

Alexander    Svpli.  u.  Auge,  1895,  22;    Cu  \'crsani.,  d.  Xat.  u.  Artz  z.  Liibeck,  189.j.  192 

Classen,  A.  f.  O..  X.  2.  l.-)7. 

Galezowski,  Annal.  d'Ociil.,  CXIV,  .389. 

Haab,   Atlas  n.  Grund.   d.   Ophthal.   88S   auf.   I-'ig.   ?,m:    Festschrift   f.    Helni'-.oltz   1891, 

Norris  &  Oljver,  ly,  528. 
Henbncr,    D    Syph.   Gefasserkrank.   d.    Gehirnarlerien,   Leipzig,    1874. 
Hirschberg,    Klin.   Beobach,    1874;     Beitr.    z.    A.,    1,   1876,   und   3,   1878;     Berlin.    Klin. 

Woch.  1888. 
Von  Hdsslin,  A.  f.  Klin.  Med.,  LXVI. 
Jacobson.   Konisberger,    Aled.    fahrb.    Bd.   1,   II.   3,  283;     Bezeih.   d.   \'erand.   u.   Krank 

d.  Sehorgans  z.  Allgemeinleiden,  24. 
Knapp,   A.  f.   A.,   IV,  205. 
Mauthner,  Lehrb.  d.  Ophthalmoscopie,  370. 

Oglesby,  Vv'ilbrand  u.   Saenger,  Die   Path.  d.   Xetz.,   T\',   1,  p.  352. 
Panas,  A.  d'O.  XXII,  69. 
Samelsohn,   Soc.   franc.   d'Oph.,   1891. 
Schcfifels,   Deutsch.   Aled.   Woch.,   1897. 
Schnbl,  Norris  &  Oliver,  ITT,  480. 

Schweigger,  Vor.   u.   d.   Gebranch   d.   Augensoiegles,   110. 
Stolting.   A.   f.   O.,  XLIII,   306. 

Retinitis  Cikcinata.  Fuchs,  in  1893,  reported  a  series  of  twelve 
cases,  presenting  a  uniformity  of  symptoms  that  entitled  them  to  be 
classed  as  an  independent  affection  which  he  designated  as  "  retinitis  cir- 
cinata."  The  condition  was  not  unknown  previous  to  Fuch's  paper,  Hutch- 
inson having  first  described  it,  in  1876,  as  "  a  symmetrical,  central,  chorio- 
retinal disease,  occurring  in  senile  persons."     Subsequently  a  number  of 


'Panas;    Samelsohn;    Alexander;    Galezowski. 


102  THE    FUNDUS    OCULI. 

cases  were  reported  as  "  Hutchinson's  changes  "  and  under  other  names.* 
The  prominent  feature  from  which  retinitis  circinata  derives  its  name 
is  a  girdle  of  white  spots  encircling  the  macula.  The  characteristic  changes 
usually  are  confined  within  the  triangular  space  enclosed  between  the 
superior  and  inferior  temporal  vessels.  In  typical  cases,  the  macula  around 
the  fovea  presents  a  greyish  opacity.  At  a  certain  distance  this  opacity  is 
surrounded  by  a  ring  of  large  and  small,  millc-white  spots.  Between  the 
ring  and  macular  opacity  the  retina  may  or  may  not  appear  normal.  Gen- 
erally, the  girdle  of  spots  is  horizontally  elliptical  in  outline  and  incomplete 
on  the  nasal  or  temporal  sides.  The  periphery  of  the  girdle  usually  falls 
short  of  the  larger  temporal  vessels  and  the  disc,  but  in  certain  cases  both 
may  be  included  within  its  radius.  The  vessels  pass  over  the  spots  when 
the  two  are  in  contact.  1  he  spots  are  not  bordered  by  pigment.  When 
first  formed  they  are  round,  but  may  coalesce  and  produce  lobulated  forms 
or  even  a  white  band  with  scalloped  edges.  The  spots  give  the  observer  an 
impression  that  they  are  slightly  elevated.  The  diameter  of  the  macular 
opacity  always  is  greater  than  that  of  the  optic  disc,  and  in  fresh  cases  it 
may  appear  sharply  defined.  Later,  the  opaque  area  may  be  extended 
and  swelling  of  the  retina  is  demonstrable.  Several  cases  have  been  re- 
ported in  which  the  retina  was  detached  in  the  macular  region.  Pigmen- 
tation does  not  belong  to  the  ophthalmoscopic  picture,  but  stippling  and  pig- 
ment spots  have  been  observed  outside  the  girdle.  Within  the  girdle,  a 
delicate  haze  hangs  over  '■he  zone  of  apparently  normal  retina.  In  old 
cases,  hemorrhages  occur  in  the  affected  region,  usually  along  the  ves- 
sels. According  to  Fuchs,  bleeding  occurs  in  about  half  of  the  cases. 
Holms  Spicer  found  hemorrhages  In  the  fellow  eye.  Fresh  spots  and 
new  hemorrhages  may  form.  In  very  old  cases  pigmentation  may 
occur.  The  optic  disc  is  unaltered  unless  touched  by  the  girdle,  in  which 
case  Its  outline  may  be  blurred.  The  retinal  veins  are  tortuous  and  the 
vessels  may  present  other  arteriosclerotic  changes.  In  course  of  time  the 
retina  included  within  the  girdle  atrophies.  The  spots  may  long  remain 
unchanged  or  may  break  up.  Fuchs  has  seen  the  macular  opacity  and 
spots  disappear  In  four  years  and  in  another  case  continue  for  seven 
years.  DeWecker  has  observed  a  case  for  twenty  years.  The  spots  some- 
times change  color  and  exhibit  a  dirty-grey  stippling,  and  cholesterine 
deposits  appear.  In  some  cases  a  proliferative  process  ensues  and  the 
retina  becomes  greatly  thickened  from  formation  of  connective  tissue. 


*Galezowski    in    1886;     Goldzieher   in    1887;     deWecker    and    Masselon    in    1891    as 
degencrescence  graisseuse;    deWecker  in   1894  as  degenerescence  blanche. 


ANGIOPATHIC    RETINITIS.  103 

Retinitis  circinata  is  a  rare  disease.  Among  70,000  eye  patients,  Fuchs 
found  eight  typical  and  four  atypical  cases.  DeWecker  saw  fifteen  cases 
among  150,000  patients.  In  Fuch's  twelve  cases,  only  one  eye  was  affected 
in  seven  and  both  eyes  in  five.  It  appears  to  be  more  common  among  women 
than  among  men.  Generally  the  subjects  are  over  60  years  of  age  and  have 
arteriosclerosis;  cases  occur,  howex'er,  at  all  ages.  DeWecker  saw  a  case 
in  an  infant.  Great  \'isual  disturbance  always  exists,  but  develops  gradu- 
ally. It  consists  of  a  relative  central  scotoma,  with  a  diameter  of  10-12 
degrees,  which  soon  becomes  absolute.  Peripheral  vision  is  not  affected, 
but  old  cases  develop  concentric  contraction  of  the  field.  The  light  sense 
remains  good.  The  patients  do  not  complain  of  night  blindness,  day 
blindness  or  metamorphopsia,  from  which  it  is  inferred  that  the  rod 
and  cone  layer  of  the  retina  is  unaffected.  The  course  of  the  disease  is 
chronic  and  progressive  and  the  prognosis  is  bad,  but  absolute  blindness 
does  not  result,  peripheral  vision  being  retained. 

The  nature  of  the  process  and  morphology  of  the  spots  have  not  been 
determined.  Fuchs  regards  the  latter  as  exudates  similar  to  those  found 
in  albuminuric  retinitis  and  denies  that  they  are  connected  with  hemor- 
rhages. Goldz.ieher  thinks  the  condition  is  a  manifestation  of  arterioscler- 
osis, the  spots  being  necrotic  areas  caused  by  closure  of  arterioles.  Xuel 
accepts  arteriosclerosis  as  the  cause  and  believes  the  spots  to  be  in  Henle's 
layer  which  is  best  developed  in  this  region.  DeWecker  declares  that  the 
spots  are  blood  clots  which  have  undergone  fatty  degeneration.  Only  one 
case,  that  of  Ammann,  has  been  microscopically  examined.  Unfortunately, 
the  authenticity  of  the  case  may  be  questioned,  as  the  patient  had  advanced 
glaucoma  and  albuminuria.  In  the  affected  part,  the  retina  had  four  times 
its  natural  thickness,  due  to  thickening  of  the  internuclear  layer.  Ammann 
decided  that  the  spots  were  clusters  of  fat  granules  and  hyaline  masses 
resulting  from  disintegration  of  blood;  but  the  blood  had  all  disappeared. 
Ammann's  conclusions  are  not  final.  Clinically,  bleeding  is  an  inconspicu- 
ous feature  in  retinitis  circinata.  When  it  occurs  it  may  or  may  not  leave 
a  white  spot,  but  there  is  no  doubt  that  the  characteristic  garland  of  spots 
occurs  independently  of  hemorrhages.  The  arrangement  of  the  spots 
around  the  macula  corresponds  to  the  terminals  of  the  perimacular  arteries, 
which  strongly  suggests  nutritive  disturbance  from  vascular  disease.  The 
gradual  development  and  progressive  character  of  the  affection  indicate 
a  chronic  degenerative  process  which,  from  its  situation,  may  be  regarded 
as  of  vascular  origin. 


104  THE    FUNDUS    OCULI. 

Stereogram  40.  Retinitis  Circinata.*  Right  eyeground  of 
woman  60  years  of  age.  In  her  first  confinement,  31  years  ago,  she  had 
puerperal  eclampsia.  Eight  subsequent  confinements  were  normal  and  all 
children  born  are  still  living  and  healthy.  Urine  normal.  Blood  pressure 
180-190  mm.  Hg.  Vision  of  the  right  eye  has  been  progressively  failing 
for  the  past  year.  Aside  from  slight  vascular  changes  in  the  retina,  the 
left  eye  appears  normal.  In  the  fundus  of  the  right  eye  is  an  open,  white 
girdle  around  the  macular  region.  The  radius  of  the  girdle  fills  the  space 
between  the  superior  and  inferior  temporal  vessels.  The  gap  in  this  circle 
occurs  on  the  side  nearest  the  nerve,  and  is  filled  by  a  yellowish-white  strip 
resembling  an  infiltration  of  the  retina.  The  girdle  is  composed  of  large 
and  small,  closely  packed,  white  spots,  sparsely  stippled  by  bluish  specks 
resembling  slight  surface  depressions.  Many  of  the  spots  have  coalesced, 
but  between  most  of  them  a  line  of  demarcation  can  be  traced.  Numerous 
small,  round,  white  dots  border  the  spots  and  coalesce  with  them.  Above 
and  on  the  outer  side  of  the  girdle  are  numerous  small,  white  and  greyish 
spots  with  diameters  approximating  that  of  the  central  vein.  Below,  in  the 
radius  of  the  white  circle,  Is  a  mass  of  branching  pigment,  lying  upon  a 
patch  of  discolored  retina.  Adjoining,  are  a  few  similar  but  smaller  spots. 
Within  the  girdle  the  entire  retina  is  opaque  and  appears  covered  by  a  grey 
haze.  The  optic  disc  contains  a  deep  physiologic  excavation  and  appears 
normal.  The  retinal  arteries  present  bright  central  light  streaks.  The 
terminal  of  the  superior  temporal  artery  runs  in  front  of  the  white  girdle 
and  In  this  part  of  Its  course  is  dilated  and  tortuous.  The  retinal  v^eins  are 
slightly  dilated.  Two  striate  hemorrhages  are  seen  along  the  course  of  the 
Inferior  temporal  vein,  Immediately  outside  the  girdle. 

Di  \gnosis.  It  is  not  difficult  to  diagnosticate  retinitis  circinata  If 
attention  is  once  directed  to  the  characteristic  arrangement  of  the  white 
spots.  In  retinitis  circinata  white  spots  never  appear  in  the  macula,  but  in 
albuminuria  and  diabetes  the  macula  Is  the  favorite  site  of  exudation. 

Bibliography. 

Amman.  A.  f.   A.  XXXV,  1807:    A.  of  Oph.,  XXVII,  1898,  203. 

DeWecker,  a.  d'Opb.  cliniqiie.  Paris,  1886. 

DeWecker-IVIasselon,  Oph.  cliniqiie,  Paris,  1891. 

Fnchs.  A.  _f.  O.  XXXIX,  3,  1893. 

Galezowski,  Traite  icon.  d'Ophthalmoscopie,  III,  Paris,  1886. 

Goldzieher,  Weiner  Med.  Woch.  1887;    B.  d.  O.  G.,  1896;    A.  f.  A.  XXXIV,  1897. 

Hutchinson.  O.  H.  Rep.,  VI  fl,  1876. 

Nuel,   A.   d'Oph.,  XVI,   1896. 

Spicer,  T.  O.  Soc,  XIV,  1894:    XVI,  1896. 


*I  am  indcl>ted  to  Dr.  Ward  S.  Holden  for  the  privilege  of  reporting  this  case 


Chapter  VIL 
CLOSURE  OF  THE  RETINAL  VESSELS. 

Closl;re  of  tiii:  Ckxtral  Artery  (Embolism).  For  the  purpose 
of  practical  discussion,  the  retinal  circulation  may  be  regarded  as  a  terminal 
system.  Such  collateral  vessels  as  exist  are  insufficient  in  size  to  maintain 
the  vitality  of  the  tissues;  consequently,  occlusion  of  the  lumeiT  in  the 
central  artery  is  immediately  followed  by  acute  retinal  anemia  and  loss 
of  vision. 

OPHTEiALMosc'oric  APPEARANCES.  If  viewed  immediately  after  the 
circulation  has  been  interrupted,  the  following  characteristic  picture  is 
presented.  The  media  are  clear;  the  fundus  has  a  dull,  slight  grey  cast; 
the  optic  disc  is  pallid;  the  arteries  are  reduced  in  size  and  the  smaller 
twigs  are  indistinguishable.  Frequently  the  arteries  appear  fairly  well 
filled  at  the  periphery  and  filliform  at  the  disc.  If  arteriosclerosis  has 
rendered  the  arterial  walls  opaque,  they  appear  as  white  cords  or  show 
irregular  constrictions  with  apparent  interruptions  of  the  blood  column. 
As  a  rule,  the  veins  can  be  traced  throughout  their  courses,  but  frequently 
their  proportions  appear  reversed,  i.  e.,  they  are  widest  at  the  periphery 
of  the  field  and  diminish  in  size  as  the  disc  is  approached. 

After  closure  of  the  central  artery  degenerative  changes  in  the  retina 
immediately  ensue.  Within  a  short  time  a  white,  cloudy,  edematous-looking 
opacity  appears  at  the  posterior  pole  and  gradually  extends  over  the  fundus. 
The  fovea  centralis,  however,  retains  its  normal  color,  appearing  as  a 
"  cherry-red  "  spot,  strongly  contrasting  with  the  surrounding  white.  The 
margin  of  the  disc  becomes  indistinct  and  surrounded  by  delicate,  radiate 
striatlons,  which  are  never  pronounced  as  is  the  case  in  tumescence  of  the 
nerve  head.  The  retinal  opacity  reveals  small  vessels  around  the  macula 
which  ordinarily  are  invisible.  At  the  periphery,  where  the  retina  is  thin- 
nest, the  opacity  gradually  disappears.  Retinal  hemorrhages,  usually  in- 
significant in  size,  are  sometimes  present  around  the  disc  and  macula.  After 
a  variable  time  (hours  or  days)  the  retinal  circulation  is,  in  a  measure, 
re-established.  When  this  first  takes  place,  a  remarkable  phenomenon, 
known  as  the  granular  current,  may  occasionally  be  observed.  In  this 
condition  the  blood  columns  of  certain  vessels,  more  commonly  the  veins, 

105 


106  •        THE    FUNDUS    OCULI. 

are  broken,  presenting  the  appearance  of  moving  red  beads  separated  by 
clear  interspaces.  The  rapidity  of  the  current  varies  and  abrupt  fluctua- 
tions may  take  place.  Thus,  the  blood  stream  may  be  moving  steadily 
through  a  vessel  when,  suddenly,  it  becomes  slower  or  even  reversed;  or 
a  to-and-fro  (pendulum)  movement  may  occur.  Rarely  all  motion  ceases. 
Pulsatory  entrance  of  blood  into  the  vessel  is  very  rare.  I  have  seen  but 
one  case.  After  circulation  is  partially  restored,  if  the  arteries  are  not 
sclerosed,  the  slightest  pressure  reproduces  the  granular  current.  Atrophy 
now  ensues.  1  he  opacity  disappears  and  the  retina  regains  its  transparency. 
The  outline  of  the  disc  becomes  sharp  and  the  nerve  atrophies.  The 
vessels -undergo  degeneration,  which  in  some  cases  is  extreme  (Stergm.  44). 
A  faint  haze  always  remains  over  the  fundus  in  the  retinal  atrophy  follow- 
ing closure  of  the  central  artery. 

Stereogram  41.*  Closure  of  the  Central  Retinal  Artery 
(Embolism?).  Left  fundus  oculi  of  a  girl  18  years  of  age;  telephone 
operator.  The  left  eye  has  been  subject  to  transitory  obscurations  of 
vision.  Suddenlv,  while  working,  she  suffered  from  slight  vertigo  and  the 
left  eye  became  blind,  but  on  this  occasion  vision  did  not  return.  The  eye 
Avas  without  light  perception  then  or  afterward.  Urine  normal;  blood 
pressure  125  mm.  Hg. ;  no  syphilis;  no  tuberculosis.  An  aortic  systolic  mur- 
mur was  heard  by  some  and  not  by  others. 

The  stereogram  was  painted  seven  days  after  closure.  The  pale 
optic  disc  is  surrounded  ?nd  obscured  by  a  broad  halo  of  opaque  retina. 
The  retina  is  opaque  also  in  the  macular  region,  strongly  contrasting  with 
the  "  cherrv-red  "  fovea.  The  larger  arteries  are  filiform  and  the  smaller 
branches  invisible.  A  few  small  hemorrhages  have  occurred  around  the 
macula  and  nerve.  The  veins  are  better  filled  than  the  arteries.  The 
supranasal  vein  is  broader  at  the  periphery  than  at  the  center.  Slight 
pressure  on  the  eyeball  produces  pulsation  In  the  vessels,  proving  that 
circulation  is  partly  re-established. 

Two  years  later  the  retina  was  highly  atrophic  (Stergm.  44).  Physi- 
cal examination  at  this  time  failed  to  reveal  any  cause  for  closure  of  the 
arterv. 

Interpretation  of  the  Ophthalmoscopic  Picture.  Retinal 
Edema.  The  intraocular  distribution  of  the  central  artery  is  limited  to 
the  inner  layers  of  the  retina;  consequently,  occlusion  of  the  artery  inter- 
rupts metabolism   in  these  layers.     The  ganglion  cells  with  their  axons 


'Patient  of  Dr.  J.  H.  Ohly.  Prooklyn   Eye  and  Ear  Hospital. 


CLOSL'Rl-:    OF    THE    RETINAL    VESSELS.  107. 

(the  nerve  fibers)  undergo  coagulation  necrosis,  whereby  their  transparency 
is  lost.  In  addition  there  is  more  or  less  serous  exudation,  analogous  to 
the  postanemic  edema  occurring  in  white  infarction  of  the  kidney,  spleen 
and  other  organs.  Ordinarily,  the  amoimt  of  edema  is  slight ;  exceptionally, 
however,  it  is  abundant  (Stergm.  42).  Retinal  opacity  usually  appears 
within  two  hours  after  closure,  rarely  later  than  the  second  day.  De 
Schweinitz  mentions  a  case  in  which  he  saw  it  20  minutes  after  an  attack. 
Von  (jiraefe's  case  was  remarkable  in  that  it  did  not  appear  for  two  weeks. 
Schweigger  regards  early  retinal  opacity  as  evidence  that  the  arterial  ob- 
struction is  nonembolic.  It  is  most  marked  around  the  disc  where  the 
nerve  fiber  layer  is  thickest  and  around  the  fovea  where  the  ganglion  cells 
are  most  numerous.  In  course  ot  time  the  waste  products  of  retinal  de- 
generation are  remo\ed,  after  which  the  inner  layers  of  the  retina  are  cor* 
verted  into  a  thin  cicatrix.  The  external  lavers  of  the  retina  are  nourished 
by  the  choroid  and  therefore  are  not  affected.  The  supporting  tissue  of  the 
retina,  which  is  common  to  all  lavers,  also  escapes  destruction. 

Stereogram  42.*  Closlre  of  Central  Retinal  Artery.  Right 
fundus  oculi  of  a  woman,  37  years  of  age.  Urine  normal.  Xo  trace  of 
syphilis.  Heart  slightly  dilated,  but  no  murmurs.  Two  weeks  ago  she 
suddenly  became  blind  in  the  right  eve,  since  which  time  there  has  been  no 
perception  of  light.  The  subsequent  history  of  the  case  is  unknown.  Oph- 
thalmoscopic examination  revealed  the  fundus  picture  of  recent  closure  of 
the  central  artery.  The  '--tina  is  opaque  throughout  the  entire  posterior 
polar  zone.  Around  the  nerve  and  at  the  macula  the  opacity  is  very 
dense,  completely  obscuring  the  disc,  while  the  "  cherry  spot  "  at  the  macula 
is  barely  distinguishable.  That  this  retina  is  edematous  as  well  as  opaque 
is  shown  by  opacities  which  overlie  the  vessels.  Arteries  are  indistinguish- 
able from  veins.  Most  of  the  vessels  taper  from  the  periphery  toward 
the  disc.  An  ascending  and  descending  vessel  appear  as  white  lines  without 
visible  blood  columns,  indicating  that  their  walls  are  sclerosed. 

FovEAL  Red  Spot.  The  ganglion  cells  and  nerve  fibers  of  the 
retina  do  not  extend  over  the  fovea  centralis:  therefore,  its  normal  red 
color  is  not  obscured  by  the  retinal  opacity  which  appears  after  closure  of 
the  central  artery,  on  the  contrary,  the  fovea  becomes  highly  conspicuous 
by  contrast  with  the  surrounding  opaque  ganglion  cells.  The  preceding 
explanation  of  the  foveal  red  spot  was  given  by  Von  Graefe  and  may  be 
accepted  as  true.     Other  less  satisfactory  theories  have  been  advanced; 


^From  the  service  of  Dr.  J.  Scott  Wood,  Brooklyn  Eye  and  Ear  Hospital. 


108  THE    FUNDUS    OCULI. 

/".  e.,  hemorrhage  (  Blessig,  Stetian)  ;  circumscribed  chorioretinitis  (Nettle- 
ship);  retinal  pigment  (Fischer);  attenuation  of  retinal  elements  and 
atrophy  of  pigment  (?:llschnig)  ;  hyperemia  of  the  choroid  (Loring).  Of 
these  theories,  only  hemorrhage  has  received  any  support.  Hemorrhages 
near  the  macula  frequently  assume  a  rounded  form  and  are  easily  mistaken 
for  the  red  fovea  (Stergm.  48).  Very  rarely,  however,  bleeding  does 
occur  in  the  fovea  (Fuchs)  in  which  case  it  may  be  regarded  a>  accidental. 
The  cherry  spot  appears  and  disappears  with  the  retinal  opacity,  which 
would  not  be  the  case  were  it  a  blood  clot.  Loring's  theory  is  supported 
by  the  case  reported  on  page  113  in  which  I  found  great  local  congestion 
of  the  choroid  behind  the  macula.  The  shape  of  the  cherry  spot  usually  is 
described  as  elliptical.  Ophthalmoscopically,  the  apparent  form  of  the 
spot  is  modified  by  position  of  the  light  and  by  shadows  cast  upon  the 
walls  of  the  macular  depression.  Anatomically,  the  fovea  is  not  oval,  but 
round.*  1  he  distinctness  of  the  spot  is  modified  by  the  degree  of  opacity 
with  which  it  is  contrasted  and  also  by  the  amount  of  retinal  edema.  If 
the  retinal  epithelium  or  choroid  is  deeply  pigmented,  the  spot  will  be 
darker  than  in  a  blond  fundus.  Inoyu  has  seen  it  coal-black  in 
a  Japanese. 

Retinal  Vessels.  Eyes  removed  during  life  or  after  death  exhibit 
arteries  diminished  in  size  but  still  containing  blood  in  Avhich  the  red  cells 
have  agglutinated  and  separated  from  the  plasma,  so  that  the  vessels 
contain  alternate  sections  of  'plasma  and  massed  red  cells  A  s'milar  con- 
dition exists  in  the  retinal  vessels  when  the  supply  of  blood  is  withheld 
from  closure  of  the  central  artery.  Those  vessels  which  appear  empty 
are,  in  reality,  filled  with  plasma.  Therefore,  it  is  inaccurate  to  describe  an 
artery  in  the  living  eye  as  empty  unless  the  lumen  is  abolished  by  collapse 
or  thickening  of  its  walls.  Arteries  filled  with  plasma  preser\'e  their  con- 
tour, appearing,  when  viewed  with  the  ophthalmoscope,  as  d'jll,  yellowish- 
tinted  cords^  differing  in  this  respect  from  an  artery  emptied  by  external 
pressure  with  the  finger,  in  which  case  the  vessel  appears  as  a  bright 
flat  hcuid.  They  differ  also  from  highly  sclerosed  vessels,  which  resemble 
white  pipe  stems.  In  cases  of  central  artery  obstruction,  if  the  retinu' 
arteries  are  not  diminished  in  size,  closure  is  incomplete.  It  is,  however, 
difficult  to  estimate  a  moderate  diminution  in  the  width  of  the  retinal  ves- 
sels. Mistakes  in  this  respect  are  frequently  made  and  are  best  avoided 
by  comparisons  with  the  fellow  eye.  The  arteries  adapt  themselves  to  a 
diminished  blood  column  by  elastic  contraction  of  their  walls   (Elschnig), 

*Piersol;     Sclimidt-Rimpler;     Merkle;     Dogiel. 


CLOSURE    OF    THE    REllNAL    VESSELS.  109 

and  also  as  a  result  of  intraocular  pressure  (lischer).  Unless  circu- 
lation is  quickly  restored,  the  vascular  contraction  becomes  permanent  from 
proliferation  of  connective  tissue  and  degenerative  changes  in  the  walls. 

Frequentlv,  the  arteries  appear  better  filled  at  the  periphery  than 
on  the  disc.  Fllschnig  thinks  this  is  due  to  the  greater  musculature  of  the 
larger  arterie?  which  enables  them  to  expel  blood  more  readilv  than 
smaller  vessels.  Reimer,  however,  states  that  the  musculature  of  a 
vessel  is  proportionate  to  its  size  and  sufficient  to  maintain  its  tone;  he 
regards  arteriosclerosis  as  a  more  probable  cause  of  the  diminished  size  of 
the  larger  vessels.  Apparently,  a  more  reasonable  explanation  than  either 
of  the  above  may  be  found  in  the  capillary  action  exerted  by  the  exceed- 
ingly small  retinal  capillaries  which,  in  the  absence  of  a  cardiac  impulse, 
would  hold  the  blood  like  a  sponge;  as  the  total  amount  of  blood  in  the 
retina  after  closure  of  the  central  artery  is  insufficient  to  till  all  its  ves- 
sels, it  accumulates  in  the  capillaries  and  the  scarcity  is  manifested  chiefly 
in  the  larger  vessels.*  Although  the  veins  are  better  filled  than  the  arteries, 
thev  frequently  are  described  as  tapering  towards  the  disc.  This  also  can 
be  explained  by  scarcity  of  blood  in  the  retina  and  its  tendency  to  remain 
in  the  capillaries,  in  the  absence  of  an  efficient  vis-a-tety/o. 

In  some  cases  of  otherwise  complete  blindness  from  closure  of  the 
central  artery,  an  area  of  light  perception  is  retained  for  a  time  on  the 
temporal  side  of  the  field.  Fischer  demonstrated  that  this  corresponds 
to  a  narrow  zone  around  the  optic  nerve,  which  is  occupied  by  capillary 
anastomoses  between  the  blood  vessels  of  the  retina  and  choroid.  This 
area  also  becomes  blind  in  the  atrophic  stage. 

In  some  eyes  the  macular  region  is  supplied  by  a  so-called  cilio- 
retinal  artery  in  which  blood  continues  to  circulate  even  though  the  main 
artery  is  closed.  When  this  condition  exists,  central  vision  alone  is 
preserved.  Wilbrand  describes  a  typical  case  in  which,  fifteen  years  after 
closure  of  the  central  artery,  a  narrow  strip  of  retina,  corresponding  to  the 
distribution  of  two  cilioretinal  vessels,  had  retained  both  normal  vision 
and  appearance  notwithstanding  all  other  parts  of  the  retina  had  undergone 
extreme  atrophy.  Cilioretinal  vessels  are  quite  common  and  are  recog- 
nized by  the  manner  in  which  thev  hook  over  the  edge  of  the  optic  disc, 
usually  on  the  temporal  side  (Stergm.  4).  They  are  supposed  to  spring 
from  the  choroidal  system  of  vessels  which  would  render  them  independent 


*In  retinitis  pigmentosa,  optic  atrophy  and  other  chronic  degenerations  of  the 
retina,  in  which  the  cardiac  impulse  is  unimpaired,  there  is  comparatively  uniform 
narrowing  of  the  retinal  vessels. 


110  THE    FUNDUS    OC'ULI. 

of  circulatory  disturbances  in  the  central  artery.  It  is  a  question,  however, 
whether  they  really  originate  in  the  choroidal  system  or  are  branches  from 
the  central  artery,  given  off  far  back  in  the  nerve,  proximal  to  the  usual 
site  of  obstruction. 

Variations  in  the  appearance  of  the  vessels  are  occasionally  noted  in 
central  artery  obstruction.  Thus,  Priestly  Smith  described  a  case  in  which 
the  veins  tapered  towards  the  disc  but  abruptly  enlarged  at  the  junction 
of  tributorv  veins,  then  again  diminished  in  size  until  joined  by  another 
tributary.  A  similar  condition  is  shown  in  stereogram  43.  Nettleship  de- 
scribes an  unusual  appearance,  seen  thirty  hours  after  closure.  At  the 
macula  the  vessels  were  bordered  by  clear  bands,  wider  than  the  vessels, 
which  were  compared  to  "  rills  of  water  in  a  dried  up  brook  channel." 
They  were  too  wide  for  degenerated  vessel  walls.  Reimer  mentions  a 
similar  appearance  in  one  of  his  cases. 

When  the  vessels  begin  to  refill  it  often  is  impossible  to  distinguish 
veins  from  arteries,  both  being  very  dark  from  deficient  oxidation  of  the 
blood  incident  to  retardation  of  the  current.  Difficulty  in  differentiation 
is  further  increased  by  the  frequent  absence  ot  light  streaks  on  the  vessels. 
In  the  atrophic  stage  the  arteries  become  more  attenuated  than  the  veins 
(Stergm.  44). 

When  obstruction  of  the  central  artery  is  at  its  height  it  is  probable 
that  a  meager  circulation,  derived  from  collaterals  around  the  nerve  head, 
still  exists  in  the  retina.  It  has  been  held  that  inability  to  produce  pul- 
sation in  the  retinal  vessels  by  external  pressure  indicates  cessation  of  the 
circulation.  This  test  is  of  little  value,  inasmuch  as  pulsation  is  not  readily 
produced  in  sclerosed  vessels,  which  is  the  usual  condition  in  closure  of 
the  central  artery. 

Clinically,  when  the  retinal  circulation  is  very  feeble,  it  is  completely 
arrested  by  the  slightest  pressure.  Remier  asserts  that  a  certain  amount 
of  circulation  is  present  so  long  as  the  blood  column  remains  unbroken, 
and  complete  interruption  can  be  assumed  only  when  the  blood  column  is 
broken  and  the  separated  portions  stand  still.  This  statement  is  founded 
upon  the  ophthalmoscopic  appearance  of  the  retinal  vessels  at  death.  It 
must  be  remembered  that  the  appearance  of  a  broken  blood  column  may 
be  simulated  by  opaque  spots  in  the  walls  of  a  vessel,  which  obscure  a 
thin  but  continuous  column  of  blood.  If  either  spontaneous  or  pressure 
pulsation  can  be  demonstrated  it  is,  of  course,  positive  proof  that  circu- 
lation exists.  The  retinal  circulation  will  be  restored,  sooner  or  later, 
after  its  apparent  suspension.     At  this  time  the  arteries  may  only  partly 


CLOSlKli    OF    THE    RETINAL    VESSEi^S.  Ill 

regain  their  former  calibre  or,  on  the  other  hand,  become  hyperdistended. 
In  the  latter  case  small  hemorrhages  occur  (Haab). 

If  blood  is  absolutely  withheld  from  the  retina,  the  ganglion  cells 
perish  in  a  very  short  time.  Under  similar  conditions  in  the  brain  the 
cells  die  within  half  an  hour  or  less.  The  clinical  histories  of  cases  in 
which  vision  has  been  completely  or  relatively  restored  several  days  after 
obstruction  of  the  central  artery,  indicate  that  sufficient  blood  was  supplied 
to  the  retina  to  preserve  tissue  vitality,  although  insufficient  to  maintain 
functional  activity.*  If  visible  degenerative  changes  in  a  retinal  artery 
have  produced  occlusion,  there  will  be  blindness  throughout  the  area  of 
its  distribution. 

Granular  Current.  When  the  blood  current  in  a  vessel  is  suf- 
ficiently retarded,  the  red  corpuscles  agglutinate  into  masses  which  float 
in  the  blood  plasma.  The  size  and  appearance  of  these  corpuscular  masses 
vary  with  the  rapiditv  of  the  current.  Thus,  if  the  blood  stream  is  com- 
paratively swift,  they  are  small,  resembling  rapidly  moving  grains  of 
sand,  while  in  a  sluggish  current  the  corpuscles  form  long,  dark  red 
cylinders,  separated  from  each  other  by  clear  spaces  occupied  by  plasma. 
The  ends  of  the  cylinders  present  a  sharply  indented  boundary  line  like  the 
fractured  end  of  a  cast  iron  bar  (Reimer).  A  similar  separation  of  the 
corpuscles  from  the  plasma  is  seen  in  the  \'essels  of  enucleated  eyes.  The 
granular  current  appears  first  in  the  veins,  where  the  blood  stream  is 
slower  than  in  the  arteries.  If  a  granular  current  appears  in  an  artery, 
while  the  corresponding  vein  presents  an  unbroken  blood  column,  the 
vein  receives  blood  from  areas  additional  to  that  supplied  bv  the  artery. 
Cessation  or  re\ersal  of  a  blood  current  in  a  xessel  mav  be  due  to  inter- 
mittent blood  supply  or,  according  to  Klschnig,  to  influx  of  venous  blood 
from  a  region  of  higher  arterial  pressure.  The  amount  of  fresh  blood 
supplied  by  a  granular  current  must  be  insignificant.  In  one  of  my  own 
cases  (Stergm.  43)  it  barely  sufficed  to  preserve  light  perception,  although, 
as  shown  by  Raehlman  in  a  case  of  extreme  arteriosclerosis  of  the  retinal 
vessels,  only  a  imall  quantity  of  blood  is  required  to  maintain  the  function 
of  the  retina. 

Hemorrh  \(iE.  In  closure  of  the  central  artery  extravasations  of 
blood  are  few  and  insignificant.  In  155  cases  of  trunk  closure  collected  by 
Fischer,  hemorrhage  occurred  in  47.  The  extravasations  are  feathery  in 
type  and  usually  appear  in  the  areas  of  capillary  anastomoses  around  the 
disc  and  macula  when  the  circulation  is  first  restored.     Haab  says  that  if 

*Hirschl)ers,  Noyes.  Alexander,  Hasse.  Perles  and  others. 


112  THE    FUNDUS    OCULI. 

the  vessels  are  hyperdistended,  bleeding  occurs,  but  it  is  uncommon  when 
the  vessels  are  incompletely  refilled.  These  observations  apply  to  sec- 
ondary hemorrhages  which  usually  appear  in  from  two  to  twelve  days  after 
arterial  closure  and  are  coincident  with  refilling  of  the  retinal  vessels. 

Thus  far,  in  this  discussion,  it  has  been  assumed  that  acute  retinal 
ischaemia  (white  infarction)  always  follows  closure  of  the  central  artery. 
While  this  is  true  for  the  great  majority  of  cases,  it  is  possible  that  under 
certain  conditions  obstruction  in  the  central  artery  may  be  followed  by  the 
picture  of  hemorrhagic  retinitis  (thrombosis).  I  know  of  no  case  in  which 
hemorrhagic  retinitis  has  been  preceded  by  the  ophthalmoscopic  picture  of 
central  artery  closure;  therefore,  if  hemorrhagic  retinitis  is  ever  caused 
by  obstruction  in  the  central  artery,  the  bleeding  occurs  at  the  onset  of  th:? 
attack;  in  other  words,  it  is  a  case  of  hemorrhagic  infarction.  Ihat  such 
an  event  ever  occurs  is  cioubted  by  Michel,  Haab,  Coats  and  Harms. 
Coats  and  Harms  accord  in  the  statement  that,  in  every  case  presenting 
the  appearance  of  retinal  thrombosis,  the  central  vein  will  be  found  ob- 
structed if  it  is  properly  examined  microscopically  throughout  its  entire 
course.  Nevertheless,  certain  cases,  particularly  one  by  Reimer,  appear  to 
prove  that,  under  certain  conditions,  hemorrhagic  retinitis  is  due  to  ob- 
struction in  the  central  artery.  1 1  denying  the  possibility  of  such  an  event, 
writers  appear  to  be  unfamiliar  with  the  experiments  of  Wm.  H.  Welch, 
who  conclusively  proved  that,  i"!  a  terminal  circulation,  hemorrhagic  in- 
farction invariably  occurs  when  the  trunk  artery  is  partially  obstructed 
and  the  blood  pressure  on  the  ciistal  side  in  front  of  the  obstruction,  falls  to 
^4  or  %  of  normal.  If,  however,  the  artery  is  suddenly  and  completely 
closed,  there  is  no  bleeding  (Stergm.  42).  It  must  be  remembered  that 
very  few  cases  of  either  central  artery  or  central  \e\n  closure  have  been 
microscopically  examineci  by  competent  pathologists;  therefore,  the  follow- 
ing authoritative  examination  by  Reimer  is  almost  conclusive. 

The  ophthalmoscopic  picture  of  narrowed  arteries,  tortuous  veins  and 
extensi^'e  hemorrhage  led  to  the  clinical  diagnosis  of  thrombosis  of  the 
central  xe'm.  Glaucoma  developed  and  the  eye  was  enucleated.  Trans- 
verse serial  sections  were  made  of  the  posterior  part  of  the  globe  and 
5  mm.  of  the  optic  nerve,  and  every  section  was  examined.  The  lumen  of 
the  central  artery  was  almost  completely  blocked  by  endarteritis  proliferans. 
The  walls  of  the  central  vein  were  thickened  but  its  lumen  was  free. 

T  am  further  strengthened  In  my  conviction  that  arterial  obstruction 
may  produce  destructive  retinal  hemorrhage  by  the  following  case:* 

*From  the  service  of  Dr.   Francis  Valk,   N.   Y.   Post  Graduate   Hospital. 


Fig.  79 — Granular  Body  of  unknown 
orii^in.  containing  Nuclear-like  Spots,  in 
the  Inner  Retinal  Layers  in  Albumin- 
uric Retinitis. 


Fifir.  79. 


Fig.  8n— Scl.-rot-c  Clian-es  in  the  Cho- 
roidal Ve  sels  in  Syphilis,  without 
marked    changes    in    the    Retina. 


Fig.  80.     f: 


Fig.  81 — Closure,  nearly  complete 
of  the  Central  Artery  by  Endarteritis 
Nodosa. 


Fig.  81. 


CLOSURE    OF    THK    RFVIINAL    MiSSELS.  113 

Unmarried  woman,  22  years  of  age.  When  Hrst  examined  ophthal- 
moscopically,  the  eye  presented  the  picture  ot  hemorrhagic  retinitis.  Later, 
intravitreous  hemorrhage  occurred,  glaucoma  developed  and  the  eye  was 
enucleated.  The  nerve  was  sectioned  transversely  throughout  the  entire 
course  of  the  central  vessels.  The  central  vein  was  not  obstructed  but  the 
lumen  of  the  artery  was  uearly  closed  by  endarteritis  nodosa    (Fig.  81). 

In  both  Reimer's  case  and  my  own  and  in  Welch's  experiments, 
hemorrhage  was  due  to  incomplete  closure  of  the  artery. 

EnoixxiY.  Closure  of  the  central  artery  usually  occurs  in  the  sub- 
jects of  cardiac  or  vascular  disease,  which  in  many  instances,  although 
present  and  progressive,  cannot  be  demonstrated.  The  majority  of  my 
cases  have  been  young  adults  in  whom  arteriosclerosis  often  is  a  local 
disease.  In  the  cases  shown  by  stereogram  41  the  only  reasonable  ex- 
planation of  the  closure  \<^as  local  disease  of  the  artery.  In  such  cases, 
further  manifestations  of  arteriosclerosis  may  be  delaved  for  years.  In 
a  girl  nineteen  years  of  age,  observed  by  Herter,  there  was  sudden  closure 
in  the  lower  main  retinal  artery.  Although  apparently  in  perfect  health 
at  the  time,  edema  of  the  feet  appeared  within  a  year.  Sometimes  the 
underlying  cause  is  undiscoverable,  as  in  a  case  cited  bv  Logetschnikow, 
in  which  closure  of  the  central  artery  occurred  in  a  girl  of  thirteen.  The 
most  exhaustive  examination  revealed  no  abnormality  to  explain  the  closure. 
Gunn  has  reported  a  similar  case.  As  a  rule,  in  closure  of  the  retinal 
vessels,  thorough  examination  will  reveal  evidence  of  vascular  disease. 
In  a  boy  17  years  of  age,  with  closure  of  the  left  central  artery.  Dr.  Glent- 
worth  Butler  found  all  surface  arteries  hardened  and  the  blood  pressure 
150  mm.  Hg.  There  was  no  cardiac  disease  and  the  urine  was  normal.* 
Aside  from  certain  exceptional  cases  closure  of  the  central  retinal  artery 
has  been  attributed  to,  {a)  arteriosclerotic  obstruction;  (7^)  thrombosis; 
{(')  embolism  and  angiospasm.  These  causes  are  supposed  to  act  singly 
or  in  combination. 

Embolisni.  In  1854  Jaeger  published  a  typical  case  of  central  artery 
closure,  in  which  he  gave  the  first  accurate  description  of  the  granular 
current,  but  he  was  unable  to  explain  the  cause  of  the  condition.  The 
phenomena  were  first  interpreted  by  Von  Graefe,  in  1859,  who  diagnos- 
ticated a  similar  case  as  embolic  occlusion  of  the  central  artery.  Although 
received  with  incredulity,  Schweigger  confirmed  the  diagnosis  two  years 
later  by  necropsy.   This  triumph  of  V.  Graefe's  genius  was  complete  and 

*Froni  Brooklyn   Eye  and  Ear  Hospital. 
8 


114  THE    FUNDUS    OCULI. 

to  many  ophthalmologists  "  embolism  "  still  suffices  to  explain  any  obstruc- 
tion in  the  central  artery.  The  chief  point  of  v.  Graefe's  discovery,  namely, 
that  the  clinical  picture  is  produced  by  closure  of  the  central  artery  of  the 
retina,  was  admitted,  but  doubts  were  soon  expressed  as  to  the  embolic 
nature  of  the  obstruction  in  all  cases.  That  these  doubts  were  well  founded 
has  since  been  demonstrated  by  microscopic  examinations,  which  show 
that  closure  usually  is  caused  by  some  process  other  than  embolism.  At 
the  present  time  nearly  all  authoritative  writers*  consider  that  in  all  cases 
so  far  reported  as  embolic  the  diagnosis  is  doubtful.  While  it  is  true 
that  an  occluding  plug  has  been  found  in  many  central  arteries,  the  de- 
ductions therefrom  must  be  accepted  with  reserve,  inasmuch  as  nearly  all 
debatable  cases  were  examined  a  year  or  more  after  closure,  at  which 
time  it  is  difficult,  if  not  impossible,  to  determine  whether  a  fibrinous  or 
calcareous  plug  was  conveyed  from  a  distance  or  developed  in  loco,  the 
great  problem  being  to  separate  the  secondary  histological  changes  from 
the  primary.  Shiba  reports  that  he  produced  embolic  closure  of  the 
central  artery  and  choroidal  vessels  by  injecting  a  quantity  of  soft  paraf- 
fin into  the  carotids.  Such  experiments  are  useful  to  demonstrate  the 
tissue  changes  following  arterial  occlusion,  but  prove  nothing  as  to  the 
probability  of  an  embolus  blocking  the  central  artery  in  man. 

Anatomical  obstacles  to  the  passage  of  an  embolus  from  the  general 
circulation  into  the  retinal  vessels  are,  the  rectangular  separation  of  the 
ophthalmic  artery  from  the  carotid  and  also  of  the  retinal  artery  from 
the  ophthalmic.  While  It  may  be  improbable  that  an  embolus  would  tra- 
verse this  route,  it  is  by  no  means  impossible.  A  more  convincing  argu- 
ment against  the  embolic  theory  is  founded  upon  certain  clinical  features 
of  the  disorder,  such  as  prodromata;  recurrent  attacks;  occasional  Involve- 
ment of  both  eyes,  or  of  both  central  vessels  in  the  same  eye;  recovery  of 
one  eye  but  not  of  its  fellow;  frequent  incompleteness  of  the  obstruction 
and  in  all  cases  re-establishment  of  the  circulation,  all  of  which  are  incon- 
sistent with  the  presence  of  an  embolus  In  a  terminal  artery.  Of  these 
objections,  discussion  has  mostly  centered  around  that  of  restored  circu- 
lation,  and  many  ingenious  theories  have  been   advanced  to  explain   re- 


*Haab,  Harms,  Hirschberg,  Raehlman,  IMichel,  Wilbrand,  Reimer  and  others. 
Harms  would  nol  diagnosticate  embolism  unless;  (1)  The  closure  occurred  from  de- 
posit of  a  body  in  the  lumen  of  a  vessel,  which  before  the  obstruction,  was  every- 
where free,  and  primary  disease  of  the  vessel  walls  could  absolutely  be  excluded; 
(2)  The  source  of  the  embolus  is  found  either  in  valvular  disease  of  the  heart  or 
other  condition  which  leads  to  thrombus  formation;  (3)  The  symptoms  indicating 
thrombotic  closure  are  not  present. 


CLOSURE    Oi-^     THE    RETINAL    VESSELS.  115 

filling  of  the  retina!  vessels  a  few  hours  or  days  after  the  central  artery 
has  been  occluded  by  an  embolus.  The  theory  of  collateral  circulation  which 
has  persistently  been  invoked  for  this  purpose,  rests  on  the  following 
anatomic  basis.  The  arterial  circle  of  Zinn  which  surrounds  the  head  of 
the  optic  nerve,  sends  branches  to  both  choroid  and  retina,  thus  establish- 
ing communication  between  these  otherwise  independent  vascular  systems. 
Unfortunately,  however,  these  collaterals  are  little  more  than  capillaries 
engaQ;ed  in  local  nutrition  and  are  wholly  incapable  of  suddenly  responding 
to  the  demand  of  an  ischemic  retina  for  sufficient  blood  to  preserve  its 
vitality.  Nettleship  has  pointed  out  that  the  location  of  a  plug  in  the 
central  artery  modifies  the  amount  of  blood  from  these  collaterals.  Thus, 
if  the  arterv  is  closed  on  the  distal  side  of  the  lamina  cribrosa  the  vessels 
from  Zinn's  circle  will  be  blocked.  On  the  other  hand,  if  the  obstruction 
is  situated  behind  the  lamina,  these  vessels  will  have  full  play.  This 
explains  certain  cases  in  which,  although  the  artery  is  closed,  the  disc 
retains  its  rosv  hue  until  the  atrophic  stage. 

Nettleship  and  Gonin  have  each  reported  cases  in  which  a  collateral 
circulation  was  ophthalmoscopically  visible  between  small  vessels  from 
the  optic  disc  and  an  obstructed  retinal  artery.  The  following  similar 
case  is  the  only  instance  I  ever  saw  of  this  phenomenon. 

Stereogr.xm  43.*  Closure  of  Central  Retinal  Artery  with 
Visible  Collateral  Circulation.  Left  eyeground  of  a  woman,  47 
years  of  age,  with  chronic  interstitial  nephritis.  The  right  eye  pre- 
sents a  typical  ophthalmoscopic  picture  of  albuminuric  retinitis.  She  has 
experienced  occasional,  transitory  obscurations  of  vision  in  the  left  eye. 
Five  hours  before  coming  under  observation  this  eye  suddenly  became  blind. 
Vision  reduced  to  perception  of  light.  The  ophthalmoscopic  picture  is  char- 
acteristic of  recent  occlusion  of  the  central  artery. 

A  typical  foveal  spot  is  situated  in  the  center  of  a  discoid  area  of 
opaque  retina.  The  optic  nerve  is  well  outlined  but  surrounded  bv  ex- 
tremely delicate  radii,  evidently  an  early  stage  of  opacity.  Elsewhere, 
the  retina  has  a  grey,  lifeless  appearance.  A  few  prominent,  white  dots, 
scattered  about  the  macular  region,  are  regarded  as  products  of  retinal 
degeneration  ciue  to  the  nephritis.  The  visible  arteries  are  highly  sclerosed 
and  appear  as  white  cords,  with  the  exception  of  the  supratemporal  branch, 
which  contains  a  sluggish  granular  current.  The  corresponding  vein  also 
exhibits  a  granular  current,  but  less  broken  than  in  the  artery.  All 
veins  appear  to  diminish  in  size  as  they  approach  the  disc.     The  blood 

*Case  was  treated  by  Dr.  S.  M.  Payne,  at  the  Manhattan  Eye  and  Ear  Hospital. 


116  THE    FUNDUS    OCULI. 

visibly  circulating  in  the  supratemporal  artery  is  supplied  by  a  plexus 
of  small  vessels,  situated  on  and  around  the  margin  ol  the  optic  disc. 
The  communication  between  artery  and  collaterals  is  plainly  seen.  On 
the  disc  the  artery  is  white  and  contains  no  visible  blood  until  joined 
by  the  newly-developed  vessels,  at  which  point  the  granular  current  com- 
mences. Pressure  on  the  globe  failed  to  produce  pulsation  but  checked 
the  circulation.  Two  days  later  circulation  was  re-established  throughout 
the  retina.  The  granular  current,  collateral  vessels  and  macular  spot  had 
all  disappeared.  Those  vessels  which,  during  the  period  of  closure,  ap- 
peared like  white  corcis,  now  exhibited  white  bordered  blood  columns. 
There  can  be  little  doubt  that,  in  this  case,  the  collateral  v^essels  came 
from  the  circle  of  Zinn  or  from  the  disc  plexus.  The  obstruction  in  the 
central  artery  of  the  left  eye  was  regarded  as  endarteritis  obliterans  which 
had  greatly  narrowed  and  occasionally  closed  the  lumen.  The  gradual 
advance  of  such  a  process  combined  with  occasional  interruptions  of  the 
circulation,  would  favor  a  high  development  of  the  collateral  vessels. 
That  the  collaterals  were  visible  may  have  been  due  to  unusual  size  or, 
as  Gonin  surmised  in  his  case,  they  were  more  superficial  than  usual.  The 
subsequent  restoration  of  circulation  throughout  the  retina  cannot  be  at- 
tributed to  the  collateral  vessels  which,  although  remarkably  developed 
could  furnish  onlv  an  insuflicient  supply  of  blood  for  a  single  arterial 
branch.  The  collateral  vessels  around  the  disc  may,  in  time,  undergo 
considerable  enlargement  but  never  develop  sufficiently  to  restore  circu- 
lation in  the  retina,  as  shown  by  the  course  of  events  when  both  retinal 
and  choroidal  supplies  are  severed  in  opticociliary  neurectomy.  In  these 
cases  the  blood  enters  the  choroid  through  recurrent  branches  of  the  an- 
terior ciliary  arteries  and,  possibly,  through  the  vena  vorticosas.  The 
new  vessels  which  then  form  around  the  nerve  head  represent  granulation 
tissue  which  soon  cicatrizes  and  obliterates  all  vessels  on  and  around  the 
disc.  In  closure  of  the  central  artery  circulation  usually  is  restored  through, 
the  regular  channels.  Schlodtmann  reported  a  case  in  which  the  optic  nerve 
and  a  nerve  tumor  were  remov-eci,  and  on  the  evening  of  the  same  ciay 
the  retinal  vessels  were  filled  with  blood.  I  have  seen  a  somewhat  similar 
case.  Of  course,  in  these  cases,  collateral  circulation  was  established 
through  new  formed  vessels  before  the  tumor  was  removed.  Coats 
suggests  that  circulation  may  be  re-established  by  enlargement  of  the  v^asa 
vasorum.  Admitting  that  such  collateral  circulation  may  be  established, 
the  interval  between  suspension  and  restoration  of  circulation  in  central 
artery  closure  is  too  short  for  such  a  system  to  develop.     In  the  case  of 


Fig.  8:; 


Closure    of    Central    Artery    with    marked    Congestion    of    the    Choroid    (c)    on    the 
Temporal   Side,  which   is  the  right  side  in  the   section. 


Fig.  83. 

The   same  as   Fig.   82,  but   shows   the   congestion   of  the    Choroid   where  it  was  most 

pronounced,    behind    the    Macula. 


CLOSURE    OF    THE    RETINAL    VESSELS.  117 

central  artery  closure  described  on  page  1  13,  there  was  marked  congestion 
of  the  choroid  on  the  temporal  side,  most  pronounced  behind  the  macula 
(Figs.  82-83).  The  engorgement  was  purely  passive.  Is  it  possible 
that  this  local  hyperemia  was  an  attempt  on  the  part  of  the  choroid  to 
supply  nutriment  to  a  starving  retina.* 

A  variety  of  explanations,  other  than  collateral  circulation,  have 
been  advanced  to  account  for  the  prompt  return  of  circulation  after 
embolic  closure  of  the  central  artery.  Schneller  suggested  an  embolus  In 
the  ophthalmic  artery  which,  after  temporarily  closing  the  mouth  of  the 
retinal  artery,  passed  onward,  permitting  the  blood  again  to  enter  the 
retina.  Mauthner  advanced  a  similar  theory.  Such  an  occurrence  would 
be  a  pathologic  curiosity,  whereas,  something  more  generally  applicable 
is  required.  There  Is  a  theory  of  incomplete  embolism,  due  to  Irregular 
shape  of  the  plug  which  permits  blood  to  leak  past  Its  edges.  Thus, 
Elschnig  describes  how  an  incomplete  embolus  grazed  the  arterial  walls 
and  provoked  a  spasm  sufficiently  strong  to  grasp  and  hold  the  foreign 
body  until  It  adhered  to  the  vessel.  When  the  spasm  relaxed  and  the 
artery  dilated,  the  embolus  remained  attached  to  one  side  only,  leaving 
an  opening  for  the  passage  of  blood.  Such  theories  are  very  properly 
rejected  by  most  investigators.!  It  Is  evident  that  an  embolus  will  be 
driven  onward  until  wedged  into  the  walls  of  the  vessel,  where  any  re- 
maining aperture  would  quickly  be  closed  by  swelling  of  the  intlma  or  for- 
mation of  a  thrombus.  The  objections  stated  apply  also  to  Wagenman's 
suggestion  that  the  plug  may  be  reduced  in  size  by  inherent  shrinking  or 
compression  from  contraction  of  the  arterial  walls.  This  idea  Ignores  the 
process  of  organization  and  union  with  the  walls  of  the  vessel  which  occurs 
in  embolism  and  thrombosis.  Fragmentation  and  distribution  of  the  em- 
bolus among  the  smaller  branches  of  the  arterv,  as  suggested  by  Fischer, 
Is  an  unusual  event  In  embolism  and,  as  pointed  out  by  HIrschberg,  the 
presence  of  such  fragments  should  produce  some  effect,  ophthalmoscopic 
evidence  of  which  is  entirely  lacking.  Canalization  of  a  soft  thrombus  or 
embolus  would  permit  the  passage  of  blood.  Although  this  process  has 
been  observed  In  a  vein,  it  has  not  as  yet  been  demonstrated  in  an  artery. 
Furthermore,     as     remarked     by     Relmer,     canalization     requires     time. 

'^Little  is  known  of  the  mechanism  of  collateral  circulation  beyond  the  fact  that 
ischemic  tissue  has  the  power  of  dilating  any  vessel  that  can  nourish  it.  If  it  be 
demonstrated  that  choroidal  A'cssels  habitually  dilate  in  closure  of  the  central  artery, 
the  impulse  to  dilation  may  arise  in  the  supporting  framework  of  the  retina  which 
is  common  to  all  layers,  and  is  nourished  by  the  choroid.  This  tissue  does  not  die 
in  closure  of  the  central  artery. 

fLeber,  Sachs,  Reimer,  Coats. 


118  THE    FUNDUS    OCULI. 

Elschnig's  view  that  an  embolus  may  be  absorbed,  leaving  no  trace  of  its 
existence,  is  unsupported  by  pathological  evidence. 

It  would  appear  that  each  case  reported  as  embolism  of  the  central 
artery  requires  the  existence  of  some  unusual  or  improbable  event  to  support 
the  diagnosis.  Nevertheless,  the  possibility  that  an  embolus  may  lodge  in 
this  vessel,  as  well  as  the  inherent  difficulties  attending  its  demonstration, 
must  be  admitted.  While  the  clinical  aspects  of  a  case  cannot  be  regarded 
as  of  much  value  in  differentiating  embolism  from  other  conditions,  it  may 
be  well  briefly  to  recall  a  historic  case.  Under  the  new  discarded  term  of 
"  infiltration  of  the  retina,"  v.  Graefe  was  familiar  with  the  ophthalmo- 
scopic picture  since  interpreted  as  "  embolism  ";*  yet  he  said  of  his  case 
that  the  fundus  picture  was  one  he  had  "  never  seen  before  in  a  case  of 
recent  blindness."  The  optic  disc  was  at  no  time  obscured  and  the  retina 
remained  perfectly  transparent  for  two  weeks  after  the  attack.  At  the 
end  of  three  weeks,  retinal  opacity  limited  to  the  temporal  side  had  so 
far  developed  as  to  biing  out  the  foveal  spot.  Schweigger  made  the 
microscopic  examination  and  found  the  central  artery  blocked  by  a  spher- 
ical coagulum^  at  the  lamina  cribrosa.  The  chief  peculiarity  of  this  case 
is  the  long  delayed  appearance  of  retinal  opacity,  a  condition  which 
Schweigger  considers  to  be  characteristic  of  embolism.  He  looks  upon 
the  early  appearing  opacity  commonly  observed  after  closure  of  the  artery 
as  an  infiltration  of-  the  retina  produced  by  gradual  slowing  of  the  blood 
current  from  endarteritis. 

In  some  cases  the  artery  is  closed  by  a  chalky  concrement  which  may 
be  an  embolus  or,  what  is  more  probable,  either  a  thrombus  or  mass  of 
endarteritic  proliferation  which  has  undergone  calcareous  degeneration. 
In  a  case  reported  by  Coats,  the  eye  was  enucleated  for  glaucoma  three 
years  after  closure  of  the  central  artery.  The  artery  was  blocked  within 
the  lamina  by  a  calcareous  mass  which  had  no  connection  with  the  walls 
of  the  ^'essel.  The  plug  was  considered  to  be  an  embolus,  a  source  for 
which  was  found  in  calcareous  deposits  on  the  aortic  valves.  In  a  case  by 
Manz,  the  patient  died  sixteen  days  after  closure.  On  microscopic  ex- 
amination a  structureless  mass,  possessing  the  staining  qualities  of  cal- 
careous matter  and  not  connected  with  the  walls  of  the  vessel,  was  found 
in  the  central  artery  at  its  bifurcation.  In  these  cases  it  was  assumed 
that  the  walls  of  the  arteries  were  too  much  degenerated  for  the  foreign 
body  to  excite  tissue  proliferation.  In  considering  these  cases  it  should  be 
borne  in  mind  that  manipulations  incident  to  enucleation  or  preparation 

*Sec   Schweiprger.  Arch,  of  Oph.  v.  XXX,  loni.  p.  506. 


CLOSURE    OF    THE    RETINAL    \  ESSELS.  119 

of  a  microscopic  slide,  frequently  displace  calcareous  masses  and  this 
sometimes  renders  it  difficult  to  determine  their  actual  location  during 
life.* 

A  source  for  the  embolus  is  seldom  found.  Kern  analyzed  95  cases 
presenting  the  clinical  features  of  embolism  of  the  central  artery  that  were 
subjected  to  autopsy.  In  66.3%  no  possible  source  for  an  embolus  could 
be  discovered,  but  a  diseased  condition  of  the  peripheral  \  essels  existed  in 
many  of  the  cases.!  Even  when  heart  lesions  are  found  it  does  not  warrant 
the  diagnosis  of  embolism,  inasmuch  as  heart  disease  and  vascular  changes 
usually  coexist.  Some  authors,  while  admitting  that  embolism  has  been 
demonstrated,  regard  it  as  an  exceptional  event.  Thus,  Coats  and  Parsons 
see  no  reason  to  doubt  that  embolism  occurred  in  the  cases  of  v.  Graefe, 
Manz,  Marple  and  Coats,  while  Schweigger  regards  the  case  of  v.  (iraefe 
as  the  only  authentic,  recorded  case.  The  majority  of  authorities,  how- 
ever, await  the  conclusive  demonstration  of  an  embolus  in  the  central  artery 
of  the  retina. 

Usually,  an  embolus  is  a  detached  piece  of  thrombus.  Occasionally  it 
consists  of  Huid,  fat  or  gas.t  Theoretically,  any  of  these  latter  could  enter 
the  retina,  but  no  such  cases  ha\e  been  demonstrated.  Schapringer  ad- 
vanced the  view  that  embolism  occurring  after  a  paroxvsm  of  coughing, 
as  in  pertussis  or  after  gastric  hemorrhag,,,  wat;  due  to  entrance  of  gas 
(air)  into  ruptured  arteries,  and  he  recommended  that  the  patient  be 
subjected  to  high  atmospheric  pressure  in  a  pneumatic  cabinet  as  is  done 
in  caisson  disease.  As  is  well  known,  incurable  blindness  may  follow  hemor- 
rhage from  the  stomach,  bowels  or  uterus,  and,  evidentlv,  is  not  altogether 
dependent  upon  the  amount  of  blood  lost.  Gas  embolism  offers  a  satis- 
factory explanation   of  such   cases. 

Angiospas.nl  When  used  in  this  connection,  the  term  spasm  should 
be  restricted  to  a  primary  contraction  of  the  walls  of  a  blood  vessel  suf- 
ficiently powerful  to  arrest  the  circulation,  the  impulse  being  imparted 
through  the  vasoconstrictor  nerves  in  response  to  a  local  or  remote  ir- 
ritation. The  term  should  not  be  applied  to  the  elastic  contractility  by 
which  the  walls  of  a  vessel  are  passively  adapted  to  a  diminished  blood 


*It  is  conceivable  that  a  calcareous  mass  may  separate  from  the  walls  of  the 
central  artery  and  clianee  its  position  so  as  to  close  the  lumen.  This  would  consti- 
tute  embolism. 

tin  63  of  these  cases,  24  had  arteriosclerosis,  5  had  chronic  nephritis  and  6  had 
syphilis. 

tF"at,  unless  in  excessive  quantity,  is  readily  dissolved  in  the  blood.  VVm.  H. 
Welch  believes  that,  in  the  living  body,  gas  may  be  generated  in  the  blood  by  the 
bacillus  areogenes  capsulatis. 


120  THE    FUNDUS    OCULI. 

column.  The  walls  of  arteries  contain  smooth  muscle  fibers  which  are  in- 
nervated by  vasoconstrictor  and  vasodilator  nerves.  In  addition,  nerves 
pass  from  the  periphery  to  the  vasomotor  centers  which  convey  centripetal 
im.pulses  capable  of  producing  the  phenomena  of  retiex  irritation.  In 
laboratory  experiments  the  vessels  of  a  part  can  be  emptied  or  distended 
by  stimulating  or  inhibiting  the  vasomotor  nerves.  On  these  grounds, 
neuropathology  recognizes  a  local  cerebral  anemia  due  to  arterial  spasm 
as  causative  for  certain  ailments,  such  as  transitory  attacks  of  aphasia, 
hemiplegia,  monoplegia,  hemianopsia,  numbness,  etc.  Among  ophthalmolo- 
gists, spasm  was  long  accepted  as  a  frequent  cause  for  retinal  ischemia, 
particularly  for  transitory  attacks  which  subsided  without  permanent  injury 
to  vision.*  Recent  critical  analysis  of  such  cases  has  thrown  great  doubt 
upon  this  theory;  in  fact,  whenever  an  opportunity  has  occurred  to  make 
a  microscopic  examination,  some  form  of  actual  obstruction  has  been 
found,  a  condition  undoubtedly  true  for  nearly  all  cases  of  acute  retinal 
ischemia.  Occasionally,  however,  a  case  occurs  in  Avhich  some  other  cause 
is  operative.  The  extreme  contraction  of  the  arteries  in  quinine  poisoning 
is  referred  to  as  an  example  of  pure  angiospasm,  but  recent  experiments  on 
animals  leave  little  doubt  that  the  contraction  is  chiefly  passive  and  results 
from  enormous  dilatation  of  the  great  splanchnic  vessels,  whereby  blood 
is  withdrawn  from  the  peripheral  areast  (Parsons).  When  blood  is 
diverted  from  the  retina  in  this  manner,  the  ischemia  is  further  increased 
by  intraocular  pressure  and  passive  elastic  contraction  of  the  arteries. 
These  observations  are  important  as  indicating  that  retinal  anemia  may 
be  produced  also  in  man  independently  of  any  obstruction  in  the  central 
artery  from  excessive  accumulation  of  blood  in  the  great  abdominal  reser- 
voirs. Probably,  tFie  retinal  ischemia  of  syncope  is  produced  in  this  man- 
ner. A  case  in  point  was  reported  by  Valaude.  A  man  36  years  of  age 
received  a  blow  over  the  heart  which  rendered  him  unconscious  for  several 
hours.  He  presented  the  ophthalmoscopic  picture  of  bilateral  ischemia  of 
the  retina.  Retinal  circulation  was  not  fully  restored  and  atrophy  followed. 
Hirschberg  first  described  cases  in  which,  after  contusion  of  the  bulb, 
blindness  of  several  minutes  duration  ensued  accompanied  by  anemia  of 


*Tn    Ole    Bull's    "  Krankheiten    der    Retinalgefiisse,"    Cramp    of    the    arteries    is    the 
dominant  pathologic  factor  throughout  the   book. 

tVascular  degenerations  and  thrombus  formation  have  been  reported  as  present 
in  the  central  artery  in  experimental  quinine  poisoning  (Emil  Behse),  De  Schweinitz 
confirmed  these  observations  but  on  re-investigation  of  his  sections  he  agreed  with 
Holden,  that  no  true  endovasculitis  or  thrombus  formation  ever  occurs  in  quinine 
poisoning. 


CLOSURE    OF     I  HE    KKTINAL    VESSELS.  121 

the  retina.  Pearse  has  reported  such  a  case  following  contusion  of  the 
cornea.  Presumably,  there  was  physical  shock  with  sudden  fall  of  blood 
pressure,  which  is  an  important  factor  in  causing  peripheral  anemia.* 
It  is  generally  conceded  that,  m  traumatic  edema  of  the  retina  (Commotio 
retmne)  concussion  is  followed  bv  retinal  angiospasm  and  ischemia  which 
soon  is  succeeded  by  vascular  dilation  and  transudation  of  serum 
(Stergm.  59).  Faravelli  tells  of  a  healthy  man  who  suffered  attacks  of 
phosphenes  followed  by  temporary  loss  of  vision,  first  in  one  eve,  later 
in  both.  During  the  seizures  the  arteries  were  reduced  to  threads,  while 
the  veins  pulsated.  After  remo\ing  a  tapeworm,  minus  the  head,  the  at- 
tacks ceased  for  two  months  but  returned  when  sections  of  the  worm 
reappeared  in  the  feces.  A  complete  and  permanent  cure  followed  re- 
mo\'al  of  both  worm  and  head.  Quaglino  describes  the  case  of  a  literary 
man  who  had  transitory  attacks  of  blindness  attended  by  sensations  of 
light  whenever  the  meal  time  was  delayed.  In  the  two  latter  cases  an 
abdominal  center  of  irritation  existed,  which  suggests  inhibition  of  the 
splanchnic  nerves  with  consequent  fall  of  blood  pressure.  Ramorino  re- 
ported two  cases  of  malarial  fe\er  in  which,  during  the  paroxysm,  blind- 
ness attended  by  sensations  of  light  occurred.  The  arteries  appeared 
filliform  and  the  veins  were  barely  perceptible.  In  the  Intervals  between  the 
attacks  the  fundus  appeared  normal.  1  he  cases  were  cured  by  quinine. 
Schnabel  describes  contraction  of  the  retinal  arteries  and  dilation  of  the 
veins  during  malarial  chill.  In  malarial  seizure,  the  accumulation  of  blood 
In  the  abdominal  viscera  suffice?  to  explain  coincident  retinal  anemia. 

For  23  years  I  had  under  observation  a  patient  who  had  occasional 
attacks  of  temporary  blindness,  during  which  the  retinal  vessels  were  re- 
duced to  threads.  The  first  attack  followed  an  almost  fatal  hemorrhage 
from  placenta  previa.  At  first,  both  eves  were  affected,  but  during  the 
last  ten  years  of  life  attacks  were  confined  mostly  to  the  left  eye.  During 
the  last  live  years  of  life  arteriosclerotic  changes  appeared  in  the  left  retina. 
Death  resulteci  from  Grave's  disease.  With  the  ophthalmoscope,  I  fre- 
quently watched  refilling  of  the  retinal  vessels.  The  first  attack  evidently 
resulted  from  exsanguinatlon.  The  cause  of  subsequent  attacks  Is  a  subject 
for  speculation. 

Cases   of   retinal   vascular  spasm   have   been   attributed  to   megrim. 


*Syncope  need  not.  as  mislit  he  supposed,  occur  in  such  oases,  inasmuch  as 
diminished  blood  pressure  is  felt  in  the.  eye  sooner  than  in  the  cranial  cavit3\  owing 
to  intraocular  pressure,  which  is  20-30  mm.  hgf.,  as  compared  with  10  mm.  hs.  in  the 
cranial  veins.     (Hall,  experiments  on  dogs.) 


122  THE    FL'NDUS    OCULI. 

Parisotti  describes  attacks  of  ophthalmo-megrim  during  which  there  was 
contraction  of  the  arteries  in  the  upper  half  of  the  fundus  with  bhndness 
of  the  corresponding  field.  Siegrist  found,  during  an  attack  of  hemicrania, 
extreme  retinal  ischenaia  on  the  affected  side.  It  may  be  remarked  that 
glaucoma  frequently  is  mistaken  for  megrim,  and  its  presence  should 
positively  be  excluded  in  making  the  diagnosis.  Ole  Bull  mistook  cause 
for  effect  when  he  said,  "  I  have  several  times  found  tension  increased 
while  (retinal)  arterial  spasm  continued."  The  appearance  of  peristaltic 
contractions  of  the  retinal  vessels,  described  by  Benson,  undoubtedly  was 
misinterpreted.  Such  spasmodic  movements  in  blood  vessels  is  unknown. 
Cases  of  sudden  blindness,  presenting  the  ophthalmoscopic  picture  of  em- 
bolism, have  followed  subcutaneous  injection  of  paraffin  into  the  nose. 
These  remarkable  cases  remain  unexplained.*  Narrowing  of  the  retinal 
vessels  is  said  to  immediately  precede  an  epileptic  seizure.! 

Great  impetus  was  given  to  the  doctrine  of  retinal  angiospasm  by 
Raynaud,  who  gave  his  name  to  a  group  of  symptoms  consisting  of 
periodic  attacks  of  either  pallor  or  cyanosis,  edema,  a  feeling  of  impending 
death  and  parasthesia  of  the  extremities  which  frequently  became  gangre- 
nous. He  attributed  the  condition  to  soasm  of  the  arteries  and,  in  his 
enthusiasm,  saw  isolated  segments  of  the  retinal  arteries  spasmodically 
contracting.  Panas,  who  ophthalmoscopically  examined  Raynaud's  cases, 
was  unable  to  see  spasm  or  other  circulatory  disturbance,  Raynaud  pub- 
lished his  ophthalmoscopic  findings  as  facts.  It  is  needless  to  say  that  these 
observations  remain  unconfirmed. 

Direct,  artificial  irritation  of  an  artery  will  induce  sufficient  spasmodic 
contraction  of  the  walls  to  close  its  lumen;  therefore,  it  is  reasonable  to  be- 
lieve that  the  irritating  effects  of  a  local  arteritis  might  also  excite  a  reflex 
angiospasm.  Such  a  case  should  be  classed  as  one  of  arteriosclerosis.  In 
view  of  the  uncertainty  as  to  the  occurrence  of  angiospasm  in  a  healthy 
retinal  artery,  supposed  spasmodic  cases  should  be  regarded  as  secondary 
to  vascular  degeneration  and  the  habits  of  the  patient  regulated  accordingly. 

Thrombosis.  Both  toxic  and  bland  thrombi  occur  in  the  central  artery. 
Toxic  thrombus  forms  in  acute  orbital  inflammation,  the  walls  of  the 
arterv  being  invaded  by  bacteria.  Bland  thrombi  are  due  to  combined 
general  and  local  causes.  General  causes  are  slowing  of  the  circulation 
and  changes  in  the  blood;  local   causes   are  sharp  bends  in  the   vessels. 


*Rohmer;  Mintz;   Hurd  and   Holden;   Moissoinnier;   Silberstein;  Uthoff. 
tKnies;    deBono;    Dollo. 


c'l.osrRK  OF   jHi:   Ri:riN.\i.  \  kssels.  123 

narrowing  or  dilation  of  the  lumen  and  changes  in  the  endothelial  lining. 
Wilbrand  and  Saenger  cite  cases  to  prove  that  thrombus  may  form  without 
coincident  arteritis,  but  Coats,  in  a  critical  analysis  of  the  same  cases, 
shows  that  such  an  event  does  not  occur.  A  thrombus  formed  in  another 
vessel  may  extend  into  a  normal  central  artery,  as  in  Siegrist's  case  where, 
following  ligature  of  the  carotid,  a  thrombus  spread  from  the  point  of 
ligature,  through  the  ophthalmic,  into  the  retinal  artery.  Analogous  cases 
are  reported  by  Michel,  and  by  Rothmund  and  Eversbusch.  Such  cases  are 
unusual  and  ha\e  little  b.^aring  on  the  question  as  to  the  frequency  with 
which  the  clinical  pictare  of  central  artery  closure  is  due  to  thrombosis. 
Reimer  regards  endirteritis  as  the  sole  factor  in  the  production  of  closure. 
He  argues  that  in  small  vessels  like  the  central  artery,  arteriosclerosis 
takes  the  form  of  endarteritis  proliferans,  a  condition  m  which,  though 
the  lumen  be  narrowed,  the  endothelial  lining  remains  smooth  and  the 
blood  is  driven  past  the  obstruction  with  increased  velocity;  whereas, 
thrombus  formation  requires  a  slow  current  and  a  roughened  surface.* 
Thrombus  formation  in  an  artery  is  uncommon  and  usually  results  from 
an  ineffective  arteritis  in  the  course  of  an  acute  fever. 

Occasional  closure  of  the  central  artery  by  combined  wall  disease 
and  thrombosis  appears  to  be  established  by  microscopic  examinations. 
The  following  case  bv  Harms  must  be  regarded  as  authentic. 

A  man  48  years  of  age,  with  hypertrophy  of  the  left  ventricle,  suf- 
fered sudden  disturbance  of  vision  in  the  left  eye.  After  three  days  the 
picture  of  macular  artery  closure  developed  with  central  scotoma.  About 
a  month  later,  rapid  obscuration  of  the  entire  field  occurred  and  24  hours 
later  the  eye  was  blind,  the  ophthalmoscope  showing  complete  closure  of 
the  central  artery.  The  eye  became  glaucomatous  and  was  enucleated. 
Microscopic  examination  revealed  sclerosis  of  the  central  artery  which 
was  plugged  at  the  lamina  by  a  well  organized  thrombus.  The  macular 
artery  and  one  other  branch  were  closed  by  thrombi. t 

The  slow,  progressiv^e  manner  in  which  closure  took  place  in  this  case 
is  considered  characteristic  of  thrombosis. 

From   the   evidence   at   hand,   thrombosis   must   be   regarded   as   the 


*Experiments  have'  demonstrated  that  clotting  of  blood  is  accelerated  by  action  of 
a  substance  furnished  by  the  walls  of  the  vessels,  which  has  received  the  name  of  throm- 
bozym  or  thrombokinase.  It  remains  to  be  demonstrated  how  this  coagulating  action  is 
modified  l^v  mural  disease.  Zarhelli  concludes  that  thrombokinase  is  not  concerned  in 
thrombus  formation,  which  is  a  process  whollv  distinct  from  clotting.  (Bernheim:  Zarhelli; 
Weil:     Wolf:     Strong.) 

tit  is  always  difficult,  often  impossible,  microscopically  to  differentiate  an  organ- 
ized thrombus  in  the  central  artery  from   endarteritic  proliferation. 


124  THE    FUNDUS    OCULI. 

final  act  in  occluding  the  central  artery  in  certain  cases  of  arteriosclerosis, 
although  it  is  far  less  frequent  than  would  be  inferred  from  English  and 
French  medical  literature. 

Arteriosclerosis  (Endarteritis  Proliferans).  In  the  preceding 
pages  it  has  been  shown  that  arteriosclerosis  is  regarded  as  the  almost 
constant  causative  factor  in  closure  of  the  central  artery.  Loring,  who 
first  advocated  this  theory,  published  in  1874  a  series  of  five  cases  pre- 
senting the  ophthalmoscopic  picture  of  embolism,  each  of  which  manifested 
some  feature  which  he  regarded  as  inconsistent  with  the  presence  of  an  em- 
bolus in  the  central  artery.* 

The  mechanism  by  which  endarteritis  is  supposed  to  close  the  cen- 
tral artery  may  be  summarized  as  follows.  At  some  point,  usually  the 
lamina  cribrosa,  progressiv^e  proliferation  of  the  intima  diminishes  the 
lumen  more  and  more  until  only  a  narrow  opening  remains.  So  long  as 
the  blood  pressure  exceeds  the  elastic  contractile  power  of  the  artery,  the 
blood  will  continue  to  flow  past  the  obstruction;  but  should  the  blood  pres- 
sure sink  sufficiently  from  any  cause,  such  as  syncope,  hemorrhage,  de- 
termination of  blood  to  other  parts,  sleep,  etc.,  or  if  the  opening  is  closed 
from  swelling  or  spasmodic  contraction  of  the  walls,  the  heart  no  longer 


*The  conception  that  closure  of  the  central  artery  might  be  due  to  vascular  diseas-^ 
has,  wrongly,  been  credited  to  Mauthner.  Mauthner  advanced  no  new  theory  to  explain 
closure;  he  simply  doubted  that  it  was  always  embolic.  He  says,  "  Tn  the  majority 
of  cases  the  pathologic  appearances  may,  perhaps,  be  ascribed  to  other  afifections  as  yet 
unknotvn."  In  a  transitory  case  he  suggested  that  return  of  circulation  niisrht  be  due 
to  fragmentation  and  distribution  of  an  embolus.  Med.  Jahrb.  d.  Wiener  Aerzte,  1873, 
S.  195:     199. 

Loring's  important  paper  embodied  all  the  arguments  employed  by  opponents 
of  embolism.  The  historic  interest  in  Loring's  paper  consists  in  the  etiologic  factors, 
other  than  embolism,  which  he  was  the  first  to  su.ggest  as  satisfactorily  explaining 
the  symptoms  presented  by  his  cases.  Heart  failure  was  ascribed  as  a  cause  for 
prodromal  attacks  of  retinal  ischemia  which  passed  away  when  the  heart  recovered  its 
tone  and  circulation  was  restored.  In  one  case  he  suggested  the  presence  of  "  some 
anatomically  different  condition  which  would  make  it  more  difificult  (in  this  case)  for 
the  heart  to  force  blood  into  the  left  eye  than  into  the  right;  hence,  recurring  blind- 
ness always  takes  place  in  the  left."  Of  another  case  he  said,  "  May  not  the  trouble 
have  been  caused  by  some  morbid  heal  process  rather  than  by  importation  of  a  plu.sj: 
Might  not  thrombosis  occur  in  the  retinal  vessels  and  might  not  this  attack  be  the  first 
expression  of  a  coming  and  more  general  trouble  in  the  minute  branches  of  the  cir- 
culatory system  at  large?"  The  paper  concludes  as  follows.  "In  the  train  of  symp- 
toms classed  under  the  title  of  embolism,  too  much  significance  has  been  laid  upon 
the  stoppage  of  the  circulation  by  importation  of  a  plug  formed  at  a  distance  from 
the  eye,  while  not  enough  stress  has  been  laid  upon  the  mechanical  actions  regulating 
the  supply  of  blood  within  the  eye,  as  well  as  upon  the  conditions  of  the  zvalls  of  the 
vessels." 

There  is  nothing  ambi.guous  in  this  language  of  Loring's  in  which  he  first  sys- 
tematically presented  the  arguments  upon  which,  he  with  Nettleship,  Noyes  and 
Priestly  Smith  founded  the  now  dominant  theory  of  a  vascular  origin  for  closure  of 
the  central  arterv. 


CLOSURE    OF     IHE    RETINAL    VESSELS.  125 

will  be  able  to  force  blood  through  the  strictured  lumen,  and  the  phenom- 
enon which  has  borne  the  name  of  embolism  then  occurs.  After  a  time  the 
blood  pressure  rises  or  the  walls  of  the  artery  recede  and  circulation  is 
restored.  The  damage  to  the  retina  will  depend  upon  the  length  of  time 
the  blood  supply  was  interrupted,  which  may  have  been  minutes  or  davs. 
This  theory  is  very  attractive  and  satisfactorily  explains  every  feature 
presented  bv  the  great  majority  of  cases.  Endarteritis  may  cause  complete 
or  incomplete  failure  of  the  retinal  circulation  and,  furthermore,  permit 
the  blood  again  to  enter  the  retina.  By  accepting  this  disease  as  the  cause 
of  obstruction  in  the  central  artery  we  are  enabled  to  understand  the  prod- 
romal obscurations  of  vision,  incomplete  closure,  sudden  return  of  cir- 
culation and  recovery  from  se\'ere  attacks.  Successive  embolic  closures 
of  one  artery  or  coincident  closure  of  iKith  central  arteries  would  require 
volleys  of  emboli,  but  there  is  nothing  unreasonable  in  the  assumption 
that  a  widely  disseminated  disease  like  arteriosclerosis  may  attack  both 
central  arteries  or  that  periods  of  exacerbation  may  occur.  In  bilateral 
attacks  reco\'ery  of  one  eye  and  continued  blindness  of  the  other  are 
explained  by  the  more  advanced  obliterative  process  in  the  blind  eye.  The 
supposed  transfer  of  an  embolus  from  a  trunk  artery  to  a  branch,  or 
from  one  point  to  another  in  the  same  artery,  is  explicable  on  the  as- 
sumption that  the  ophthalmoscopic  appearance  of  a  migratory  embolus 
was  produced  by  se>eral  points  of  cndarteritic  obstruction  at  which  the 
blood  column  was  temporarily  arrested. 

In  some  cases  microscopic  examination  has  failed  to  reveal  a  plug  in 
the  central  artery  although  the  ophthalmoscopic  picture  indicated  that 
this  vessel  was  closed.*  The  microscopic  evidence  of  endarteritis  as  the 
cause  of  closure  does  not,  howexer,  depend  upon  failure  to  discover 
an  embolus  in  the  artery,  but  upon  the  interpretation  of  histological 
changes  found  in  an  actual  obstruction. 

Un^usual  Causes.  Beal  reports  a  closure  of  the  central  artery 
following  violent  compression  of  the  neck  and  thorax  {traumatic  as- 
phyxia, p.  75).  At  first  there  was  total  blindness  but  eight  days  later 
the  patient  could  count  fingers.  The  fundus  presented  a  typical  picture  of 
"  embolism."      The  case  terminated  in  optic  atrophy. 

Neuberger  saw  retinal  ischemia  follow  a  punctured  wound  of  the 
orbit.  Von  Michel  microscopically  examined  a  traumatic  case.  The  eye 
was   injured   by   a   cane.      Profuse   subjunctival   hemorrhage   and   exoph- 


•"Loring.  Popp,  Hirschberg,  Michel  and  Uthoff. 


126  THE    FUNDUS    OCULI. 

thalmus  followed.  The  fundus  presented  the  characteristic  picture  of 
central  artery  closure.  Six  weeks  later  the  patient  died  from  tetanus. 
Near  the  optic  foramen,  a  piece  of  wood  was  found  embedded  in  fetid 
pus.  A  hyaline  thrombus  filled  the  lumen  of  the  central  artery  from 
its  origin  at  the  ophthalmic  to  its  bend  after  entering  the  nerve. 

It  has  been  suggested  that  the  central  artery  frequently  is  closed  by 
external  pressure  from  various  causes,  such  as  tumors,  swelling  of  re- 
trobulbar neuritis,  hemorrhage  into  the  nerve  or  nerve-sheaths,  etc.  In 
most  cases  such  pressure  would  interrupt  circulation  in  the  flaccid  vein 
rather  than  in  the  rigid  artery  and  the  result  would  be  stasis,  not  anemia. 
It  is  not  infrequent,  however,  for  the  vein  to  leave  the  nerve  shortly 
after  passing  out  of  the  eyeball,  the  artery  traversing  the  nerve  alone  for 
a  distance  of  several  mm.  In  such  cases  it  is  evident  that  pressure  upon 
the  nerve,  behind  the  exit  of  the  vein,  would  act  only  on  the  artery.  Such 
a  condition  may  have  existed  in  Schweigger's  case,  in  which  a  patient  with 
unilateral,  retrobulbar  neuritis,  suddenlv  became  blind  in  the  affected  eye. 
The  fundus  presented  the  picture  characteristic  of  "  embolism."  On  the 
evening  of  the  same  dav  the  arteries  refilled  and  vision  improved.  Seggel* 
reported  the  case  of  a  soldier  whose  left  eye  suddenly  became  blind  from 
closure  of  the  central  artery.  Death  soon  followed  from  pneumonia. 
The  autopsy  showed,  on  the  distal  end  of  the  nerve,  a  point  of  firm 
adhesion  between  the  nerve  and  its  sheaths.  Corresponding  to  this  place,  a 
small,  firm  tumor  was  found  in  the  nerve.  The  growth  was  diagnosticated 
as  gliosarcoma. 

In  a  few  cases  blindness  with  the  ophthalmoscopic  appearance  of 
central  artery  occlusion  has  followed  blunt  force.  In  the  cases  of 
Nettleship  and  Oeller  it  resulted  from  a  blow  on  the  orbital  bones.  The 
probable  cause  was  compression  of  the  retinal  artery  near  the  ophthalmic 
by  a  fractured  bone. 

Closure  of  the  central  artery  from  hemorrhage  into  the  nerve  sheaths 
or  into  the  substance  of  the  nerve  (Magnus)  is  unsupported  by  clinical  or 
microscopic  evidence. 

AxATOMTCAi  Characters.  In  the  optic  nerve,  the  wall  of  the 
central  retinal  artery  consists  of  three  coats,  /.  e.,  adventitia,  media  and 
intima.  Of  these,  the  intima  is  of  the  greatest  pathologic  importance. 
The  adventitia  is  not  separable  from  the  connective  tissue  framework  of 
the  nerve.  •  The  media  is  composed  of  fibrous  and  elastic  tissue  containing 
a  few  muscle  fibers.  The  intima  consists  of  three  layers,  (1)  a  single 
layer  of  endothelial  cells  lining  the  lumen;   (2)    a  layer  of  subendothelial 


CLOSURE    OF     THE    RETINAL    VESSELS.  127 

tissue;  (3)  a  homogeneous  elastic  membrane,  the  Icini'nia  elastica  intcrua, 
also  called  crenated  membrane  because  it  falls  into  folds  when  the  artery 
is  emptied.*  Nearly  all  the  morbid  changes  in  the  central  artery  occur 
in  the  intima.  As  the  retinal  artery  enters  the  eye  it  parts  with  its  sub- 
endothelial  tissue  and  elastic  membrane.  The  latter,  however,  is  re- 
placed by  elastic  libers. 

Occlusion  of  the  central  artery  usually  occurs  within,  or  near,  the 
lamina  cribrosa.  Exceptionally,  closure  occurs  in  other  parts  of  its 
course.  In  a  case  by  Hirschberg  the  artery  was  closed  just  beyond  its 
origin  from  the  ophthalmic,  outside  the  dural  sheath.  In  Velhagen's  case  a 
plug  was  found  at  the  point  where  the  artery  passed  through  the  dural 
sheath.  In  the  region  of  the  lamina  cribrosa  the  walls  of  the  retinal 
vessels  are  more  subject  to  disease  than  in  any  other  part  of  the  nerve 
or  retina.  The  reason  for  this  is  obscure,  although  it  is  probable  that  the 
anatomy  of  this  region  is  responsible.  As  the  central  vessels  pass  through 
the  lamina  they  are  somewhat  narrowed!  and  firmly  enclosed  by  con- 
densed connective  tissue.  This  constriction  at  the  terminal  end  of  the 
artery  acts  as  a  nozzle,  giving  added  impetus  to  the  blood  stream  just 
where  it  encounters  resistance  from  intraocular  pressure.  A  weak  heart 
may  be  unable  wholly  to  overcome  this  resistance  and  thus  the  blood 
mav  whirl  and  eddy  at  this  point,  a  condition  considered  as  sufficient  to 
establish  either  a  proliferative  process  or  thrombus  within  the  artery. 
Another  factor  is  the  abundance  of  perivascular  tissue  in  this  region, 
which,  like  connective  tissue  elsewhere,  frequently  becomes  the  seat  of 
chronic  inflammation.  Haab  has  suggested  that  the  movements  of  the 
eyeball,  by  dragging  on  the  optic  nerve,  may  provoke  irritation  where 
it  is  inserted  into  the  globe. 

An  embolus  excites  inflammation  in  the-  walls  of  the  artery  both  in 
front  and  behind  its  point  of  impact    (Harms). 

Toxic  thrombi  usually  are  of  the  red  or  hyaloid  variety. |      It  Is  the 


*The  name,  "  elastic,"  which  is  applied  to  this  tissue  is  unfortunate  and  leads 
to  confusion,  especially  when  considering  the  blood  vessels  whose  elasticity  and 
contractility  are  apt  to  be  attributed  to  the  action  of  clastic  tissue.  As  truly  stated 
by  Coats,  there  is  no  proof  that  this  tissue  is  elastic,  in  fact,  it  exhibits  no  elasticity 
as  illustrated  by  the  internal  elastic  membrane  which  becomes  crenated  on  collapse 
of  the  artery.  Furthermore,  when  the  number  of  elastic  fibres  in  the  walls  is  in- 
creased  the   vessel   loses   all    elasticity   and   becomes   rigid. 

tThe  normal  central  artery  measures  145  ix  at  the  lamina  and  166  p.  1  mm.  behind 
it       (Coats.) 

JWhen  the  blood  is  filled  with  pathogenic  organisms,  bacterial  emboli  may  lodge 
in  the   central   artery   and   produce   occlusion.      Friichte   reports   a   case    of   sepsis   with 


128  THE    FUNDIS    OCULI. 

bland,  white  thrombus  which  is  supposed  to  close  the  central  artery  in 
those  cases  formerly  considered  as  embolic.  Unfortunately,  the  cases 
so  far  reported  were  not  microscopically  examined  until  the  characteristic 
histologic  structure  of  a  thrombus  had  been  lost  in  the  process  of  organi- 
zation. If  an  embolus  or  thrombus  occludes  the  central  artery,  it  is  soon 
replaced  by  cells  and  tissue  proliferated  from  the  intima  which  are  difficult 
to  distinguish  from  similar  ceils  and  tissue  produced  by  a  primary  endar- 
teritis. This  difficulty  is  increased  after  degenerative  changes  are  estab- 
lished, at  which  time  the  original  nature  of  the  obstruction  cannot  be  cie- 
termineci  (Figs.  89-92).  It  is  possible  that  calcareous  or  tissue  masses 
resulting  from  endarteritic  proliferation,  may  sometimes  separate  from 
the  walls  of  the  central  artery  and  plug  the  vessel  beyond  the  point  of 
their  origin. 

A  peculiarity  of  arteriosclerosis  is  its  frequent,  apparent  limitation 
to  a  system  of  vessels  or  even  a  single  vessel.  As  a  rule  the  walls  are  af- 
fected for  short  distances  only,  uniform  thickening  throughout  the  course 
of  an  artery  being  rare.  Nearly  all  the  morbid  changes  in  the  central 
artery  occur  within  the  circle  of  the  internal  elastic  membrane  under  the 
form  of  endarteritis  obliterans  or  proliferans.  The  early  histological 
changes  occurring  in  closure  of  the  central  artery  from  endarteritis  have 
not  hitherto  been  reported.  According  to  Ribbert  and  Jores,  the  most 
important  preliminary  process  that  takes  place  in  arteriosclerosis  is  dis- 
tension of  the  tissue  and  formation  of  spaces  in  the  intima.  Ribbert  sum- 
marizes his  views  as  follows:  "As  a  result  of  increased  blood  pressure, 
fluid  constituents  from  the  blood  are  forced  into  the  intima,  separating 
the  fibrilhe,  dilating  the  spaces,  and  causing  the  fibrillated  basement 
membrane  to  swell."  In  the  following  case  the  microscopic  findings  con- 
form with   Ribbert's  description.* 

The  patient  was  a  man,  5  1  years  of  age.  i\s  demonstrated  at  autopsy, 
he  had  chronic  endocarditis,  pericarditis  and  pleurisy.  The  immediate 
cause  of  death  was  edema  of  the  lungs.  Two  weeks  before  death  the 
left  eye   suddenly  became  blind.     The   fundus  presented  the  ophthalmo- 


ulcerative  endocarditis  in  which  closure  of  the  centra!  artery  occurred.  Lonaitudinal 
section  of  the  nerve  showed  the  lumen  filled  with  a  homogeneous,  stratified  mass, 
which  Friichte  considered  an  embolus,  but  others  might  regard  as  septic  hyaline 
thrombus. 

*  From  the  service  of  Prof.  Francis  Valk,  N.  Y.  Post  Graduate  Hospital.  T 
have  withheld  and  studied  this  case  for  many  years  and  the  diagnosis  was  made  only 
after  the  most  careful  research  and  consultation.  Among  others.  Profs.  T.  Mitchell 
Frudcn   and   Henry  T.    Brooks   concur   in   the   histologic   findings   described   above. 


Fia-  84. 

Closure  of  Central  Artery — Lonjitudinal  Section. 

The  endothelial  lining  of  the  artery  is  compressed  into  a  string  of  cells  by  serous 
exudate.  In  front  of  the  occlusion  thus  formed,  to  the  left  in  the  figure,  the  lumen  is 
filled   with    blood    not    possessing   the    anatomic    structure    of    thrombus. 


Fig.  85. 

Closure  of  Central  Artery  by  Active  Endarteritis. 

The  artery  is  shown  to  the  right;  the  vein  to  the  left. 


CLOSURE    OF    THE    REThNAL    VESSELS.  129 

scopic  picture  typical  of  an  embolus  in  the  central  artery.  The  eye  and 
optic  nerve  were  removed  at  autopsy  and  sent  to  me  for  examination. 
The  nerve  was  cut  longitudinally.  Complete  serial  sections  were  made 
and  every  section  examined. 

Immediately  behind  the  lamina,  the  lumen  of  the  central  artery  was 
closed  by  swelling  of  the  intima.  The  swelling  was  due  to  a  serous 
exudate  in  the  spaces  of  the  subendothelial  tissue,  which  were  so  dilated 
that  the  endothelial  lining  of  the  artery  was  compressed  into  a  string  of 
cells  (Fig.  84).  The  fluid  which  filled  the  subendothelial  space  was 
slightlv  fibrillated  and  contained  a  number  of  leucocytes  but  no  red  cells. 
Immediately  in  front  of  the  occlusion  the  lumen  of  the  artery  was  filled 
by  old  "  stagnant  "  blood  which  did  not  possess  the  anatomic  structure  of 
a  thrombus.  Behind  the  occlusion  the  artery  was  almost  completely  col- 
lapsed and  contained  no  blood  corpuscles.  7  he  obstruction  bore  no  re- 
semblance to  the  endothelial  detachments  produced  by  rough  manipulations 
(Fig.  90).  Certainly,  the  exudate  and  leucocytes  in  the  subendothelial 
tissue  were  not  artefacts.  In  the  later  stages  of  endarteritis,  two  types 
of  obstructive  proliferation  occur  in  the  central  artery:  (1)  cellular  or 
cellulofibrous  proliferation;  (2)  increase  in  the  elastic  tissue.  These  two 
processes  frequently  coexist.  Mucous  swelling,  atheromatous  softening, 
and  ulceration  do  not  occur  In  the  central  artery  of  the  retina. 

The  following  case  is  an  exquisite  example  of  the  cellulofibrous  type 
of  endarteritis  :* 

Man,  44  years  of  age,  with  advanced  general  arteriosclerosis. 
Entered  the  clinic  with  hemorrhagic  retinitis.  Glaucoma  developed  and 
the  eve  was  enucleated.  Two  weeks  later  violent  hemorrhage  occurred 
from  the  stump. 

The  entire  optic  nerve  was  sectioned  transversely  and  every  section 
examined.  The  walls  of  the  central  vein  were  greatly  thickened  and  com- 
pletely collapsed.  7  he  lumen  of  the  central  arterv  was  closed  by  active 
endarteritis  (Figs.  85-86).  In  this  case  it  Is  difficult  to  determine  on  which 
side  of  the  vascular  system  occulsion  first  occurred.  This  difficulty  is 
further  increased  by  extreme  angiosclerosis  of  the  retinal  vessels.  Within 
the  limits  of  the  lamina  elastlca  there  is  an  exuberant  proliferation  of  large 
vesicular  cells  (Fig.  86).  Although  epithelioid  In  type,  these  cells  are 
not  regarded  as  offsprings  of  the  endothelium  but  of  the  subendothelial 
tissue    (Coats).     They  either  sw^ell   and  disintegrate   or  become  fibrous. 


*From  the  clinic  of  Dr.    J.   Scott   Wood,   Brooklyn   Eve  and   Ear   Hospital. 
9 


130  THE    FUNDUS    OCULI. 

The  cut  shows  a  mass  of  tissue  formed  in  this  manner,  situated  betwee:i 
the  endothelial  lining  and  nest  of  xesicular  cells.  The  uniform  appearance 
of  the  cells  inciicates  that  all  are  of  nearly  the  same  age.  This  fact  sug- 
gests that  an  eruptive  prolileration  of  such  cells  would  close  the  artery, 
and  later,  through  shrinkage,  the  lumen  might  reopen.  In  this  as  in 
all  similar  cases,  proliferation  proceeds  from  one  side  of  the  artery,  pro- 
ducing an  eccentric,  irregular  lumen.  Frequently  the  cellulofibrous  tissue 
assumes  a  nodular  form  which  projects  into  the  lumen  usually  from  one 
side  only,  rarely  from  both  (Pig.  81).  In  either  case  the  lumen  is  re- 
duced to  a  slit-like  opening.  At  first  the  nodes  consist  cf  cells  but  later 
the  mass  is  invaded  by  elastic  and  fibrous  connective  tissue,  which  replace 
the  cellular  elements   (Fig.  88). 

More  or  less  increase  of  elastic  tissue  in  the  intima  occ-irs  in  all  types 
of  endarteritis,  but  sometimes  proliferation  of  this  tissue  is  so  excessive 
as  to  constitute  the  sole,  or  at  least  principal,  cause  of  arterial  obstruction. 
The  vessels  shown  m  Fig.  87  are  from  a  case  of  general  arteriosclerosis. 
The  section  was  made  through  the  anterior  part  of  the  lamina  cribrosa  and 
stained  by  Weigert's  method  for  elastic  tissue.  Both  branches  of  the 
central  vein  are  completely  closed  by  cellular  and  fibrous  tissue.  The 
intima  and  media  of  the  central  artery  are  replaced  by  thick  layers  of 
elastic  tissue.  As  a  rule,  the  media  takes  no  part  in  the  sclerosing  process. 
Occasionally,  however,  as  in  this  case,  concentric  narrowing  of  the  lumen 
occurs  from  proliferation  of  elastic  tissue  in  the  outer  coats  which  replaces 
the  muscularis  as  well  as  the  intima.  In  the  illustration,  notice  the  entire 
absence  of  elastic  fibers  in  the  veins,  which  proves  that  these  vessels  have 
already  assumed  their  retinal  structure.  In  the  following  case  the  central 
artery  was  entirely  replaced  by  a  cord  of  elastic  tissue. 

Male,  32  years  of  age.  Operation  on  the  frontal  sinus  was  followed 
by  orbital  cellulitis.  T  saw  him  first  at  this  time,  in  consultation  with  Dr. 
Lewis  Cofl'in.  O.  D.  Exophthalmus  was  excessive.  Cornea  hazy.  No 
perception  of  light.  Blindness  occurred  suddenly.  Details  of  fundus  were 
obscured  by  condition  of  the  cornea.  Pupil  dilated  and  immobile.  Vit- 
reous clear.  The  fundus  had  a  uniform,  dirty-white  reflex.  No  retinal 
hemorrhages.  View  of  the  vessels  was  unsatisfactory.  An  opinion  was 
given  that  the  central  artery  was  closed  by  septic  thrombosis.  Incision  of 
the  orbital  tissue  evacuated  pus  and  the  exophthalmus  gradually  subsided. 
The  eye  never  recovered  perception  of  light.  Ulceration  and  cicatriza- 
tion of  the  cornea  prevented  further  examination  of  the  fundus.  Three 
months   later  the  eye   was   enucleated.      Transverse   serial   sections   were 


Fig-.  86 — Closure  of  Central  Ar- 
tery by  Endarteritis.  The  Artery  is 
to  the  left  in  the  Figure.  Within 
the  Elastic  Lamina,  which  is  Cren- 
ulated  and  Deeply  Stained,  a  Pro- 
liferation of  Larsre,  Vesicular  Cell- 
has    taken   place. 


Fig.  86. 


Fig  87 — Complete  Closure  of  both 
Tiranches  of  the  Vein  (to  the  right) 
by  Fibrous  Tissue.  The  Artery  (to 
the  left)  is  Narrowed  by  Prolifera- 
tion of  the  Media,  its  Elastic  Lamina 
is  Deeply  Stained  by  Weigert's 
?,rcth.oH. 


FiR.  87. 

Fig.  88— The  Obstruc- 
tion caused  by  Endarter- 
itis has  been  Converted 
into  Fibrous  Connective 
Tissue  in  the  Vein  (to 
the  left)  and  into  Elastic 
Tissue,  which  is  Deeply 
Stained,  in  the  Artery, 
shown  to  the  right.  The 
Vein  ib  hardlj'  Distin- 
guishable from  the  Sur- 
rounding Connective 
Tissue. 


Fig.  88. 


Fig.  89. 

Occlusion   of   the    Central   Artery. 

Degenerative  changes  render  impossible  the  determination  of  the  true  nature  of 

the   obstruction. 


Fig.  90. 

Occlusion   of  the    Central   Artery. 

The   Endothelial   Detachment  is   an   artefact  produced   in   the   preparation   of  the 
specimen.     The  elastica  (deeply  stained)  has  been  stripped  from  the  walls. 


CLOSURi:    OF     THE    RETINAL    VESSELS.  131 

made  of  the  posterior  part  of  globe  and  optic  nerve.  Both  central 
artery  and  vein  were  completely  obliterated  in  and  behind  the  lamina 
cribrosa.  The  two  vessels  were  enclosed  in  a  common  sheath  which  was 
moderately  infiltrated  with  round  cells.  The  artery  consisted  almost 
whollv  of  elastic  tissue  (Fig.  88)  and  the  vein  of  organizing  connective 
tissue  and  cells.  The  retina  was  edematous  and  its  inner  layers  degen- 
erated. No  evidence  of  intraocular  hemorrhage  was  found  except  some 
pigment  granules  on  the  disc. 

A  frequent  artefact  in  the  central  artery,  often  mistaken  for  a  patho- 
logical process,  is  produced  during  enucleation  by  dragging  and  torsion 
of  the  nerve  and  especially,  by  unsuccessful  attempts  to  sever  the  nerve 
with  scissors.  A  great  variety  of  conditions  result,  but  in  all,  the  distin- 
guishing feature  is  a  fresh,  normal  condition  of  the  blood  and  tissue 
elements.  Usually  the  lumen  of  the  artery  is  blocked  by  massed  blood 
corpuscles  and  spindle  cells.  The  endothelial  layer  may  be  shredded  or 
the  elastica  stripped  from  the  walls  and  entangled  in  the  blood  (Fig.  90). 
Collections  of  fresh  blood  may  occupy  the  intima.  Cases  reported  as  de- 
tachment of  the  intima,  exfoliation  of  endothelium,  dissection  of  the 
walls  by  blood  and  fresh  thrombi  are  examples  of  this  artefact.  The  onlv 
case  of  active  endothelial  proliferation  and  loosening  which  I  have  seen 
was  also  due  to  traumatism,  but  in  this  case  it  is  believed  to  have  occurred 
before  enucleation. 

L.  i\l.,  healthy  mechanic  27  years  of  age.  The  left  eye  was  per- 
forated by  a  piece  of  steel  which  passed  through  the  ciliary  bodv  and  lodged 
in  the  tissues  at  the  inner  border  of  the  papilla.  Intraocular  hemorrhage 
was  slight.  Four  hours  later  the  steel  was  located  by  skiography  and  re- 
moved by  magnet.  Mild  cyclltis  developed  and,  fearing  sympathetic  oph- 
thalmitis, the  eye  was  enucleated  on  the  fourth  day  after  injurv-  Micro- 
scopic examination  showed  no  evidence  of  purulent  infection.  The  former 
site  of  the  foreign  body  was  distant  about  1  mm.  from  the  central  artery. 
There  was  round  cell  infiltration  of  the  surrounding  parts  but  the  tissue 
process  appeared  to  be  reparative  rather  than  inflammatory.  The  follow- 
ing interesting  changes  were  noted  in  the  central  artery.  Distension  of 
the  perivascular  lymph  sheath  and,  on  the  side  of  the  vessel  adjacent  to 
the  injury,  there  was  edematous  thickening  of  the  media.  At  this  point  the 
endothelial  cells  were  loosened  and  increased  in  numbers,  forming  a  loosely 
adherent  mass  which  projected  across  the  lumen.  Adjoining  this  mass, 
were  single  cells,  partly  detached  and  adherent  to  the  walls  by  pointed  tips, 
their  long  diameters  extending  out  into  the  lumen  (Fig.  91). 


132  THE    FUNDLS    OCULI. 

In  endarteritis,  the  progressive  deposit  of  new  tissue  finally  Is  ar- 
rested by  nutritive  disturbance  and  retrogressive  alterations  ensue.  If, 
through  these  processes,  the  central  vessels  are  occluded,  the  products 
of  proliferation  and  degeneration  may  be  removed  and  all  traces  of 
the  vessel  obliterated  by  encroachment  ol  the  surrounding  connective 
tissue   (Fig.  92) . 

Clinical  History.  Prodomata,  consisting  of  transitory  obscura- 
tions of  vision,  which  may  be  alarming  or  so  slight  as  to  be  forgotten  by 
the  patient,  usually  are  present.  At  last,  sudden  blindness  ensues  which 
Is  permanent.  Transitory  attacks  may  continue  for  twenty  years  or  longer 
with  no  apparent  Injury  to  vision.  In  other  cases,  permanent  blindness 
may  follow  the  first  seizure.  An  attack  usually  is  unattended  by  subjective 
svmptoms,  although  vertigo,  faintness  and  photopsia  have  been  observed. 
In  rare  Instances  both  eyes  are  affected  simultaneously  or  at  different 
times.*  In  Hasse's  case  the  interval  was  ten  years.  In  bilateral  cases 
the  eyes  mav  be  affected  in  varying  degrees.  After  a  severe  attack  most 
cases  advance  to  complete  blindness,  even  though  temporary  improvement 
may  take  place.  In  a  case  observed  by  Sichel  there  was  at  first  great  Im- 
provement, but  four  months  later  vision  again  sank  to  bare  perception  of 
light.  If  the  blindness  Is  absolute  during  an  attack,  the  prognosis  Is  bad. 
If  central  scotoma  exists,  useful  vision  cannot  be  expected.  At  first  the 
whole  field  may  be  darkened  and  later  a  quadrant  clear  up.  Even  in  the 
most  favorable  cases  peripheral  contraction  of  the  visual  field  is  to  be 
expected.  A  prognosis  of  useful  vision  may  be  given  in  cases  where 
central  vision  is  preserved.  Despite  the  unfavorable  outlook  for  most 
cases,  a  number  have  been  reported  in  which  vision  was  nearly  or  com- 
pletely restored.! 

A  few  cases  have  occurred  and  been  microscopically  examined,  in 
which  closure  of  the  artery  was  followed  by  unilateral  glaucoma.  In  all 
but  three  of  these  the  increased  tension  was  explained  by  pathological 
conditions  other  than  closure  of  the  artery.|  Closure  of  the  central  artery 
is  not  very  rare,  and  three  cases  occurring  in  connection  with  glaucoma 
constitute  too  small  a  percentage  to  establish  a  causative  connection  be- 
tween the  two  conditions.     It  is  held  that  arteriosclerosis  is  the  common 

'*•  Hirschberg,   Ewers,   Hasse,   Benson,   Blagowjestschenski. 

t  Uthoff ,  Raelilmann,   Mooren,  Olaf  Page,  Coats. 

t  Cases  of  Nettleship,  Manz  and  Marple.  Nettleship's  second  case  is  not  in- 
cluded, owing  to  lack  of  correspondence  between  the  clinical  and  microscopic  findings. 
The  onset  of  blindness  was  attended  by  severe  pain  and  the  ophthalmoscopic  picture  Vvas 
not  characteristic  of  "  embolism."     Probably,  the  pathological  examination  was  not  complete. 


Fig.  91. 


Fig.  92— Degenerative  Changes  fol- 
lowing Obstruction  and  Obscuring  the 
Nature  of  its  Origin.  All  Traces  of  the 
Vessel  have  been  Obliterated  by  Sur- 
rounding  Connective   Tissue. 


Fig.  91 — Edothelial  Proliferation  due 
to  Traumatism.  The  Perivascular  Lymph 
space  is  Distended,  the  Media  Thickened, 
and  the  Endothelial  Cells  are  Increased 
in-  Numbers  and  Project  across  the 
Lumen. 


Fig.  92 


Fig.    93 — Retina    Two  Weeks 

after  Closure  of  Central  Artery. 

The    Inner    Layers    are  Degen- 
erated  and    Edematous. 


Fig.  93. 


CLOSURE    OF    THE    RETINAL    VESSELS.  133 

cause  of  both  the  central  artery  closure  and  subsequent  glaucoma.  Were 
this  true,  the  order  of  events  occasionally  would  be  reversed  and  closure 
of  the  artery  follow  glaucoma;  such  an  occurrence,  however,  appears  to 
be  unknown.*  Primary  glaucoma  is  regarded  as  a  bilateral  affection. 
In  these  cases  the  glaucoma  was  limited  to  the  eye  in  which' the  artery  was 
closed,  indicating  that  it  was  secondary  to  some  morbid  condition  in  the  af- 
fected eye.  That  cessation  of  retinal  circulation  would  directly  affeci  intra- 
ocular pressure,  is  inconceivable;  therefore,  the  development  of  glaucoma, 
probably  depended  upon  some  local  process  which  was  not  ascertained. 
It  may  be  possible  that,  inasmuch  as  the  inner  layers  of  the  retina  die 
after  closure  of  the  central  artery,  the  products  of  their  decomposition  may 
excite  sufficient  reaction  in  the  pectinate  ligament  to  establish  a  glaucoma- 
tous tendency.  It  has  not  been  established,  however,  that  such  a  tendency 
exists. 

Diagnosis.  Recent,  well  de^■eloped  cases  of  central  artery  closure 
can  hardly  be  mistaken  for  any  other  condition.  If  the  retinal  opacity 
is  verv  grear,  it  may  completely  obscure  the  nerve.  Such  cases  are  dif- 
ferentiated from  choked  disc  by  the  small  veins  and  absence  of  swelling; 
also  by  the  macular  spot  and  by  amaurosis.  The  foveal  red  spot  is  nearly 
diagnostic.  It  may  de\elop  also  in  comotio  retina?,  but  in  this  condition  the 
vessels  are  not  reduced  in  size.  Amaurotic  idiots  show  a  similar  appear- 
ance at  the  macula,  but  there  is  no  opacity  around  the  nerve  and  the  retinal 
vessels  are  normal.  .Amaurotic  idiocy  occurs  only  in  infants.  A  macular 
hole  sometimes  appears  in  the  retina  after  injury,  especially  in  myopic  eyes. 
In  these  cases  peripheral  vision  is  preserved,  while  in  closure  the  entire 
field  is  darkened.  Small  macular  hemorrhages  are  usually  round  and 
may  resemble  the  red  spot  of  "  embolism."  After  circulation  is  resumed 
and  \ision  partlv  restored,  the  diagnosis  rests  largely  on  the  history.  At 
this  time,  slight  pressure  on  the  globe  with  the  finger  may  arrest  retinal 
circulation  and  render  visible  obscure  mural  opacities.  In  old  cases  of 
closure  in  which  circulation  has  been  resumed,  concentric  contraction  of 
the  visual  fields  usually  exists. 

OccuLSiON  Atrophy  (Atrophic  Stage  of  Central  Artery  Closure). 
If  the  central  artery  is  once  completelv  blocked  by  endarteritis  obliterans, 
embolus  or  thrombus,  the  inner  lavers  of  the  retina  and  the  optic  nerve 
undergo  extreme  atrophy  (Figs.  93-94). 


*  Ridley's  case,  which  Haab  quotes  as  an  example,  was  one  of  hemorrhagic  glaucoma, 
in  which  wide  st3read  vascular  disease  existed. 


134  the  fundus  oculi. 

Stereogram  44.  Retinal  Atrophy  Following  Closure  of  the 
Central  Artery.  Left  fundus  oculi  of  girl  who  two  years  ago  had 
complete  closure  of  the  central  artery  of  the  retina.  The  ophthalmoscopic 
picture  which  the  eye  presented  at  that  time  is  shown  in  stereogram  41. 
Vision  never  was  restored  after  closure  first  occurred.  Externally,  the 
eye  appears  normal.  Light  perception  is  absolutely  abolished.  Direct 
pupillary  reaction  to  light  is  lost  but  consensual  reaction  is  normal.  The 
fundus  picture  is  characteristic  of  atrophy  confined  to  the  inner  layers  of 
the  retina.  There  is  no  pigmentation  or  other  evidence  that  either  the 
outer  lavers  of  the  retina  or  the  choroid  are  in  any  way  disturbed.  The 
atrophic  nerve  has  a  bluish  cast  but  the  markings  of  the  lamina  cribrosa 
are  not  visible,  as  is  the  case  in  simple  optic  atrophy.  Only  two  arteries 
contain  blood  columns,  the  others  being  reduced  to  white  bloodless  cords  or 
entirely  obliterated.  The  veins  which  remain  are  highlv  sclerosed,  but  con- 
tain more  blood  than  the  arteries.  A  delicate  veil,  representing  the 
atrophic  layers  of  the  retina,  hangs  over  the  entire  fundus. 

Diagnosis.  Occlusion  retinal  atrophy  is  recognized  by  its  extreme 
character,  absence  of  postinflammatory  products,  pigmentation  or  hemor- 
rhage and  bv  absolute  blindness. 

Branch  Artery  Closure.  When  a  branch  of  the  central  artery 
of  the  retina  is  obstructed,  the  same  changes  occur  within  the  area  of  its 
distribution  as  in  obstruction  of  the  trunk  artery  and  a  scotoma,  cor- 
responding to  the  affected  region,  appears  in  the  visual  field.  It  has  been 
stated  that  the  macular  branch  is  most  frequently  closed.  Probably,  this 
is  more  apparent  than  real,  inasmuch  as  a  small,  peripherally  situated 
scotoma  may  escape  detection,  whereas,  a  central  defect  is  noticed  at 
once.  Closure  of  a  sub-branch  causes  a  fan-shaped  scotoma  highly  char- 
acteristic of  the  condition.  The  retinal  opacity  is  more  prominent  in 
branch  closure  than  in  closure  of  the  central  artery,  from  contrast  with 
the  adjacent,  unaffected  retina.  Occasionally,  edema  is  excessiv^e.  Hirsch- 
berg,  describing  such  a  case,  said:  "  Fhroughout  the  area  of  distribution 
of  the  obstructed  artery  the  retina  was  whitish  and  edematous.  To  those 
unacquainted  with  the  picture  it  might  be  mistaken  for  detachment  of  the 
retina,  a  mistake  that  appears  sometimes  to  have  occurred."  It  must  be 
remembered  that  the  retinal  opacity  soon  passes  away,  after  which  the 
visible  alterations  may  not  correspond  with  the  functional  disturbance. 
At  this  period  perimetric  measurements  will  reveal  the  characteristic  sco- 
toma of  branch  closure,  thus  indicating  the  real  trouble.  By  the  same 
means  it  may  be  possible  to  distinguish  a  primary  vascular  degeneration, 


CLOSURE    OF    THP:    RETINAL    VESSELS.  135 

from  a  vascular  degeneration  secondary  to  closure  of  an  artery.  Bleeding 
usually  is  absent  in  closure  of  a  branch  artery.  An  associated  branch  artery 
and  vein,  however,  may  both  be  closed  from  endovasculitis  or  thrombosis, 
in  which  case  hemorrhages  and  edema  occur  throughout  the  region  of 
their  distribution.  The  possibility  of  hemorrhagic  infarction  from  in- 
complete closure  of  a  branch  retinal  artery  is  a  subject  for  discussion 
(see  p.  112).  Reimer  considers  branch  closure  usually  to  be  incomplete 
because  a  broken  blood  column  has  never  been  observed  on  the  peripheral 
side  of  the  obstruction. 

The  point  of  obstruction  being  ophthalmoscopically  visible  in  branch 
closure,  the  latter  has  been  subjected  to  the  most  careful  scrutiny.  Many 
observers  have  seen  what  they  believed  to  be  an  embolus  blocking  the 
artery.  These  obstructions  have  been  variously  described  as  "  dark-red  en- 
largements," "  white  or  grey  lines,"  or  as  "  white  scales  on  the  vessels," 
etc.  Sometimes  the  plug  has  appeared  to  change  its  position,  spontaneously 
or  from  external  pressure.  Frequently,  they  have  disappeared  altogether. 
The  more  complete  knowledge  acquired  regarding  the  ophthalmoscopic  ap- 
pearances produced  by  arteriosclerosis  makes  it  more  than  probable  that 
nearly  all  so-called  emboli,  seen  with  the  ophthalmoscope,  in  reality,  were 
illusory  effects  incident  to  changes  in  the  walls  of  the  arteries.  Thus, 
sclerotic  spots  in  an  otherwise  transparent  vessel,  or  transparent  places 
in  a  sclerosed  vessel,  or  a  point  of  obstruction  at  which  the  blood  column 
was  arrested,  were  misinterpreted  if  the  mind  of  the  observer  was  pos- 
sessed by  the  embolic  theory.  A  conical  pointing  of  the  blood  column  at 
each  end  of  the  supposed  embolus  has  often  been  described,  a  condition 
now  known  to  be  pathognomonic  of  narrowing  of  the  lumen  from  thicken- 
ing of  the  walls.  Raehlmann,  speaking  of  the  ophthalmoscopic  appearance 
in  arteriosclerosis,  says:  "  In  former  years,  when  I  first  observed  these 
changes,  I  thought  1  had  before  me  the  remains  of  an  embolic  process." 
From  the  evidence  at  hand  it  must  be  concluded  that  so-called  branch 
embolism  is,  in  the  great  majority  of  cases,  due  to  obstruction  of  the  blood 
stream  from  thickening  of  the  arterial  walls. 

Stereogram  45.  Closure  of  Branch  Retinal  Artery.  Married 
woman,  30  years  of  age.  Mother  of  two  healthy  children.  Has  never  mis- 
carried. Wassermann  test  for  syphilis  and  Yon  Pirquet  for  tuberculosis, 
negative.  Urine  contains  a  trace  of  albumin.  Blood  pressure  125  mm.  Hg. 
Dr.  Glentworth  Butler,  who  made  a  physical  examination,  found  neither 
cardiac  nor  vascular  disease.  Four  days  ago  sudden  blurring  occurred 
in  the  left  eye.     Light  perception  is  lost  In  the  upper-inner  visual  field  (Fig. 

I 


136  THE    FINDUS    OCULI. 

95).  With  the  ophthalmoscope  a  retinal  opacity  is  seen  covering  the 
infratemporal  quadrant  of  the  fundus,  most  dense  in  the  macular  region. 
On  the  temporal  side,  the  opaque  and  normal  retina  are  sharply  separated 
by  ;i  wavT  line  which  passes  directly  through  the  fovea  centralis.  On  the 
infranasal  side,  the  white  opacity  fades  gradually  into  the  normal  red 
fundus.  The  opaque  region  is  triangular  in  form  with  the  apex  at  the 
disc.  The  lower  half  of  the  fovea  is  situated  within  the  opacity  where  it 
appears  as  a  semicircular  red  spot,  continuous  above  with  the  normal 
macula.  The  affected  region  represents  the  area  of  distribution  of  the 
infratemporal  retinal  artery  which  is  obstructed  at  the  disc.  At  its  exit 
from  the  nerve  this  vessel  is  opaque  for  a  distance  of  about  one  disc 
diameter.  Within  the  opaque  portion  of  the  artery  are  two,  short,  rzd 
segments  of  visible  blood  column.  Branches  of  the  artery  distributed  to 
the  macula  are  rendered  visible  by  contrast  with  the  opaque  retina.  The 
infratemporal  vein  contains  blood  but  is  broader  at  the  periphery  than  at 
the  disc.  Evidently,  the  arterial  occlusion  is  incomplete.  Pressure  with 
the  finger  on  the  eye  fails  either  to  empty  the  occluded  artery  of  blood  or 
produce  pulsation. 

Diagnosis.  Closure  of  a  branch  artery  often  escapes  discovery.  It 
may  be  mistaken  for  flat  detachment  of  the  retina  or  edema  due  to  effusion 
from  the  choroid.  In  detachment  both  arteries  and  veins  are  full,  darkened 
and  somewh?.t  tortuous,  while  in  arterial  occlusion  the  vessels  are  straight 
and  narrowed.  The  surface  of  a  detachment  always  is  somewhat  elevated, 
which  is  not  the  case  in  occlusion.  Good  light  perception  or  even  vision 
is  retained  in  recent  detachment,  but  the  area  affected  by  closure  of  an 
artery  is  blind.  Moreover,  the  triangular  form  of  opacity  in  branch  closure 
is  diagnostic  of  the  condition.  An  isolated  patch  of  opaque  retina  from 
choroidal  effusion  (Stergm.  18)  is  differentiated  from  the  opacity  of  vessel 
closure  by  its  elevated  surface,  normal  or  congested  vessels,  its  form  and 
situation,  which  bear  no  relation  to  an  artery  and,  in  nearly  all  cases,  the 
presence  of  other  choroidal  lesions.  PhotopsicT  are  a  symptom  of  choroid- 
itis but  are  not  present  in  closure  of  the  retinal  vessels.  Circulation  may  be 
restored  after  branch  closure,  but  the  scotoma  may  remain.  The  triangular 
shape  of  such  scotomata  indicates  their  v^ascular  origin. 

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Strong,  The   Laryngoscope,  St.   Louis.  Feb.,   1911. 
Uthoff,   -Berlin    Klin.    Woch.    1890,    825    and    1906.    626;    27    Berich.    d.    Heid.     Oph. 

Gesell.  p.  121. 
Valaude,  Annal.  d'Ocul.,  CXIT.  266. 
Velhagen.   ref.  in    Tahresb.   f.   Oph.   1905.  240. 
Von   Graefe.   A.    f.    Oph.,   V..    1859,   p.    136. 
Von  Michel,  Zeit.  f.  A.,  XXI,  1909,  116. 
Wasgenmann,  A.  f.   O.  XLTV,  2.  1897,  219  and  XLIV.  3;    XXXVI.  4.  1890.  p.   1; 

XXXVIII,  3,  1892,  p.  213;  XL,  3,  1894,  p.  221. 
Weil,  Presse  Med.,  Oct.  1905. 
Welch,  Trans  Assn.  Am.  Physicians,  1887;    and  in  Albutt  and  Rolleston,  VI,  1'  GO, 

p.  762. 
Welt,  A.   f.  A.,  XLI,  335;    A.  of  O.  N.  Y.,  XXX.  1901,  495. 

Wilbrand  u,  Saenger,  IV,  Path.  d.  Xetz    1909,  v.  273,  also  vol.  Ill,  p.  664-786. 
Zarhelli,  Beitrag.  z.  path.  Anat.  u.  z.  alleg.  Path.,  XLVII,  3,  1910,  p.  539. 

Closure  of  the  Central  Vein  of  the  Retina  (Thrombosis). 
When  the  central  vein  of  the  retina  is  occluded,  the  conditions  existing  in 
closure  of  the  central  artery  are  reversed.  Instead  of  retinal  ischemia, 
a  state  of  static,  venous  engorgement  ensues,  the  intensity  of  which  depends 
uDon  the  degree  of  obstruction  in  the  vein.  Michel,  who  first  demonstrated 
the  condition,  separates  it  into  three  classes:  (1)  Complete  obstruction; 
(2)    Nearly  complete  obstruction;    (3)    Partial  or  slight  obstruction. 

In  cases  of  the  first  class,  namely,  complete  obstruction,  the  fundus 
presents   the   picture   of   extreme   hemorrhagic    retinitis.      The    veins    are 


CLUSLRE    OF    THE    RETINAL    VESSELS.  139 

enormously  distended,  irregular  in  size  and  very  tortuous,  the  sinuosities 
being  mostly  perpendicular  to  the  plane  of  the  retina,  rising  above  the 
surface  in  black,  knuckle-like  loops  and  then  disappearing  into  the  swollen 
retina  or  pools  of  blood.  The  arteries  are  more  or  less  diminished  in 
size,  according  to  the  amount  of  pressure  exerted  upon  their  walls  by 
swelling  of  the  nerve.*  The  arteries  about  the  disc  frequently  are  obscured 
by  blood  and  exudates.  The  vessels,  especially  the  veins,  may  be  bordered 
by  white  lines  due  to  wall  degeneration  although  tortuosity  and  bleeding 
are  not  great  when  the  veins  are  highly  sclerosed.  White  borders  to  the 
veins  may  be  due  also  to  etiusion  into  the  perivascular  lymph  channels 
(perivasculitis) . 

The  retina  is  covered  with  flame-shaped  or  round  hemorrhages,  most 
numerous  around  the  disc,  where  the  blood  may  form  a  continuous  sheet. 
As  a  rule,  the  blood  does  not  break  through  the  limiting  membrane  of  the 
retina  into  the  vitreous.  Areas  of  white  exudation  abound,  especially 
where  sharp  bends  occur  in  the  veins.  The  macula  exhibits  a  grey  dis- 
coloration unless,  as  is  frequent,  it  is  the  site  of  hemorrhage.  There  is 
always  more  or  less  redness  and  edema  of  the  nerve-head.  Usually  the 
swelling  is  very  slight,  but  occasionally  is  great  enough  to  be  classed  as 
neuritis  ( hciuorrJiagic  neuroret'uiitis) .  I  have  usually  found  the  nerve 
swollen  when  retinal  thrombosis  was  associated  with  albuminuria.  Red- 
ness of  the  ner^•e  likewise  is  variable,  sometimes  slight  and  again  sufl'icient 
to  obliterate  the  outline  of  the  disc.  If  the  central  vein  is  occluded,  the 
veins  upon  the  disc  cannot  be  emptied  by  external  pressure  upon  the  globe, 
although  an  intermittent  blood  current  can  be  thus  produced  In  the  arteries, 
indicating  that,  notwithstanding  closure  of  the  trunk  vein,  circulation 
through  the  venous  collaterals  in  the  nerve  still  exists.  In  a  case  of  Bal- 
laban's,  the  veins  could  not  be  emptied  by  pressure,  but  circulation  was 
manifested  bv  the  unusual  feature  of  a  granular  current  in  the  veins. 

In  cases  of  the  second  class,  In  which  nearly  complete  obstruction 
exists,  the  veins  are  equallv  dilated  but  hemorrhages  are  less  numerous 
than  in  the  first  class.  Probably,  the  veins  upon  the  disc  can  be  emptied 
by  external  pressure. 

In  cases  of  the  third  class,  where  obstruction  is  comparatively  slight, 
the  veins  still  are  quite  tortuous  and  the  disproportion  in  size  between 
them  and  the  arteries  is  very  apparent.  Hemorrhage  is  slight  or  mav 
not  occur.  The  veins  on  the  disc  can  be  seen  to  empty  when  pressure  is 
applied  to  the  eye. 

*Reimer  attribute?  the  shrinking  of  the  arteries  to  obstructive  endarteriti-^. 


140  THE    FUNDUS    OCULI. 

Wilbrand  and  Saenger  state  that  bleeding  in  venous  obstruction  may 
be  slight  under  the  following  conditions:  (a)  extreme  weakness  of  the 
heart;  (b)  incomplete  closure  of  the  vein;  (r)  closure  of  both  central  artery 
and  vein;  (d)  when  cilioretinal  veins  are  present.* 

In  the  early  stage  of  venous  closure  the  retinal  exudates  have  a 
milky,  edematous  appearance,  due  to  large  admixture  of  serous  transudate. 
The  dense  white  patches  which  appear  a  few  days  later  are  connected  with 
the  processes  of  blood  resorption. 

Stereogram  46.  Closure  of  Central  Retinal  Vein.  (Retinal 
Thrombosis;  Retinitis  Hemorrhagica;  Apoplexy  of  the  Retina).  Mu- 
latto woman,  45  years  of  age.  Laundress.  Urine  normal.  Blood  pres- 
sure, 250  mm.  Hg.  Surlace  arteries,  rigid.  Numbness  of  left  hand  and 
leg.  Five  weeks  ago,  on  w^aking  in  the  morning,  she  discovered  that  her 
left  eye  was  blind.  At  the  present  time,  with  this  eye,  she  can  see  mov^e- 
ments  of  the  hand.  The  ophthalmoscope  shows  the  entire  fundus  covered 
with  blood.  Most  of  the  hemorrhages  are  striate  but  many  are  deep  and 
round.  An  almost  continuous  sheet  of  blood  extends  around  the  upper 
and  temporal  sides  of  the  disc.  The  nerve  head  is  not  swollen,  but  is 
covered  and  obscured  by  a  patch  of  white  exudate.  Similar  masses  of 
exudate  are  scattered  throughout  the  fundus.  The  enormously  dilated 
and  tortuous  veins  are  either  entirely  buried  in  the  swollen  retina  or  rise 
above  the  surface  in  dark,  venous  loops.  The  arteries  are  also  obscured 
by  the  swelling  and  hemorrhage,  but  wherever  visible  they  do  not  appear 
narrowed.  The  veins  cannot  be  emptied  of  blood  by  external  pressure  on 
the  eve. 

Diagnosis.  Only  the  condition  known  as  thrombosis  of  the  central 
vein  of  the  retina  uroduces  the  picture  shown  by  this  stereogram.  From 
choked  disc  it  is  differentiated  hv  the  extensive  bleeding  and  absence  of 
nerve  swelling.  Hemorrhage  from  traumatism  would  be  more  local  or, 
if  extensive,  blood  would  appear  in  the  vitreous.  That  venous  obstruction 
in  the  above  case  is  complete  is  shown  by  the  amount  of  blood  extrava- 
sated  and  by  inability  to  empty  the  veins  on  the  disc  by  external  pressure 
with  the  finger. 

Etiology.  Aside  from  albuminuric  cases,  closure  of  the  central 
vein  usually  occurs  in  elderly  people  v/ho,  although  apparently  in  good 
health,  in  reality  are  subjects  of  cardiac  or  vascular  disease.  The  disorder 
Is  prone  to  attack  those  who  have  passed  their  lives  in  laborious  toil,  espe- 


*The  existence   of  cilioretinal   veins   is  doubtful    (page    16). 


CLOSLRE    OF    THI-:    KK'ilXAl.    \  KSSELS.  141 

daily  in  a  stooplnij;  position.*  The  disease  is  more  common  among  men 
than  women  which  may  be  attributed  to  the  more  v'iolent  exercises  or 
intemperate  habits  of  the  former,  conditions  which  lead  to  degenerative 
changes  in  the  heart  and  blood  vessels.  The  most  frequent  systemic 
change  noted  has  been  arteriosclerosis,  rigidity  of  the  surface  arteries 
being  recorded  in  most  reports.  Old  age  predisposes  to  the  affection. 
Thus,  among  54  cases  tabulated  by  Harms,  44  were  over  forty  years  of 
age.t  The  average  ratio  of  predisposing  and  associated  diseases  is  il- 
lustrated in  50  cases  collected  by  Moses  of  complete  obstruction  in  the 
central  vein  or  some  of  its  branches.  Among  these,  38  had  arteriosclerosis. 
The  majority  were  over  50  years  of  age.  In  those  below  this  age,  other 
morbid  conditions  coexisteci  with  vascular  disease.  For  example,  5  were 
diabetic,  4  had  contracted  kidneys  and  2  pernicious  anemia.  Two  cases 
showed  struma  and  mitral  insufficiency.  In  one  case  the  heart  was  dis- 
placed by  kyphoscoliosis.  The  influence  of  vascular  disease  upon  the 
retinal  circulation  was  illustrated  in  a  case  where  the  central  vein  was 
closed  in  one  eye  and  the  central  artery  in  the  other.  In  another  case  of 
closure  the  fellow  eye  exhibited  the  picture  of  albuminuric  retinitis. |  In  clo- 
sure of  the  central  Aein,  disease  of  the  retinal  vessels  is  usuallv  found  on 
microscopic  examination,  although  the  degenerative  changes  mav  not  have 
been  sufficientlv  advanced  to  produce  visible  opacity  of  the  walls  when 
viewed  w'th  the  ophthalmoscope.  In  Schonewald's  18  cases,  retinal  angio- 
sclerosis  was  ophthalmoscopicallv  obser\ed  in  nine.  Svphilis  is  rarely 
mentioned  as  a  causativ^e  factor,  but  should  be  carefullv  sought  for  when 
retinal  thrombosis  appears  in  young  people.  Nephritis  and  diabetes,  by 
producing  vascular  degeneration,  are  causes  of  thrombosis  but  when,  as 
is  common  in  these  diseases,  thrombosis  occurs  in  a  branch  vein,  it  is 
associated  with  such  wide-spread  inflammatorv  and  exudative  conditions 
that  the  ophthalmoscopic  picture  is  classed  as  retlntis  of  albuminuric  or 
diabetic  origin.  Thrombosis  is  not  uncommon  after  influenza  and  in  a 
considerable  proportion  of  cases  it  forms  in  the  central  vein.§  In  other 
microbic  and  exhausting  diseases  thrombosis  of  the  central  vein  is  rare, 
(marantic  thrombus).     Wiirdemann  saw  a  case  following  mumps,   in   a 

*Among  Amman's  20  cases.  1?.  were  accustomed  to  .gardening  and  2  were  smiths. 
Haab   also   mentions   posture   as   exerting  an   influence   upon   thrombus    formation. 

tGoh's   case   of   septic   thrombosis   is   omitted. 

irSchonewald  collected  18  cases  of  thrombosis  of  the  central  vein.  There  was 
Jieneral  arteriosc'erosis  in  7,  grave  heart  disease  in  R  and  pulmonary  emphysema  in  2. 
Four  developed  glaucoma  and  one  died  from  cerebral   apople.xia. 

§Three  of  Coat's  16  cases  followed  grip.  Tn  cases  of  intraocular  trouble  follow- 
ing grip,  the  accessory  nasal  sinuses  should  be  carefully  examined. 


142  THE    FUNDUS    OCULI. 

boy  eight  years  of  age.  Goh  examined  the  eyes  of  a  man  25  years  of  age 
who  died  from  "  sepsis  hemorrhagica."  There  was  a  hyaline  thrombus 
in  the  right  central  vein.  Tn  the  left  eye,  several  retinal  vessels  and  a 
choroidal  vein  were  closed /by  thrombi. 

Thrombosis  is  favored  by  certain  changes  in  the  composition  of  the 
blood,  particularly  anemia.  In  pernicious  anemia,  cases,  are  reported  by 
Moses  and  Michel.  Both  Bondi  and  Michel  have  seen  cases  in  leukemia. 
The  one  serious  complication  of  chlorosis  is  thrombosis  and  sometimes 
this  occurs  in  the  central  vein  (Clermont,  Ballaban).  Haab  has  observed 
retinal  thrombosis  in  the  intense  anemia  of  pregnancy.  iVlichel  thinks  that 
incomplete  thrombosis  of  the  retinal  vein  is  common  in  the  cachectic 
anemia  of  malignant  growths. 

In  orbital  cellulitis  closure  of  one  or  both  central  vessels  occurs  from 
thrombosis  or  proliferating  vasculitis.  These  cases  are  attended  by  '"'- 
tense  inflammation  and  exophthalmus.  They  are  of  microbic  origin  and 
originate  in  accessory  sinus  disease,  facial  erysipelas,  wounds  of  the 
orbit,  etc. 

An  abnormal  course  of  the  central  \'ein  predisposes  to  sclerosis  and 
thrombus  formation.  In  25%  of  the  cases  examined  by  Harms  the 
central  vein  formed  loops  before  emerging  from  the  nerve  sheath.  Tor- 
tuosity of  the  vein  also  is  described  by  Goh,  Welt,  Yamaguci  and  Coats. 

Other  conditions  which  have  been  supposed  to  cause  thrombosis  of 
the  central  vein  are:  suppression  of  menses;  menopause;  cessation  of 
customary  hemorrhoidal  bleeding;  extensive  burns  of  the  skin;  poisoning 
by  phosphorous  and  by  nitro-benzol.  In  the  condition  last  named  the 
fundus  presents  a  violet  color  and  the  blood  vessels  appear  Inkv-black. 

A  few  cases  haxe  been  reported  of  central  vein  closure  in  young 
people,  especially  women,  m  whom  neither  circulatory  or  other  systemic 
change  could  be  found.  Coats  mentions  such  a  case  in  a  young  woman 
who  recovered  norm.al  vision.  Parsons  reported  a  case  in  a  young  woman 
with  irregular  menstruation,  who  recovered  with  vision  of  6/9.  I  have 
observed  a   similar  case. 

Stereogram  47.  Obstruction  of  Central  Vein  with  Neuritis 
(Hemorrhagic  Neuroretinitis) .  Unmarried  woman,  28  years  of  age. 
Menses  commonly  delayed  three  or  four  months.  Right  eye  normal. 
Left  eye  vision  20/200.  Aside  from  menstrual  disorder  nothing  abnormal 
could  be  discovered  by  the  most  exhaustive  examinations. 

Fundus  Conditions.  The  optic  disc  is  edematous  and  slightly 
swollen.    The  height  of  the  swelling  does  not  exceed  2  D.  (.66  mm.).   The 


CLOSURE    OF    TH1-:    RETINAL    VESSELS.  143 

entire  retina  is  edematous  and  covered  with  scattered,  striate  and  round, 
hemorrhages.  The  veins  are  dilated  and  tortuous,  especially  the  ascend- 
ing branches.  The  arteries  are  somewhat  obscured  by  retinal  edema  but 
otherwise  they  appear  normal.  Pressure  on  the  eye  empties  all  veins  on 
the  disc  with  the  exception  of  the  ascending  temporal.  The  appearance  of 
the  veins  on  the  disc  after  recovery,  indicated  that  the  obstructive  path- 
ologic process  was  anterior  to  the  lamina  cribrosa  and,  probably,  involved 
the  ten)poral  veins  after  they  emerged  from  the  nerve.  This  situation 
of  the  obstruction  (phlebitis  or  thrombus)  may  have  caused  the  nerve 
to  swell.  The  hemorrhages  were  gradually  absorbed  and  vision  was  re- 
stored to  20/30  where  it  has  remained  for  the  past  eight  years.  The 
appearance  of  this  fundus  after  recovery  is  shown  in  Stereogram  49. 

Dl-xcnosis.  In  this  case  the  nerve  is  swollen  but  not  enough  to  pro- 
duce hemorrhages.  Here,  the  amount  of  bleeding  and  retinal  edema  is 
greater  than  would  occur  in  any  obstruction  due  to  mere  swelling  of 
the  nerve.  Pressure  on  the  eve  empties  the  veins  indicating  an  incom- 
plete closure  of  the  central  \ein  of  the  retina,  i.  e.,  a  thrombus  of  the 
second  class. 

Anatomical  Characters.  It  has  been  demonstrated  that,  in  the 
great  majoritv  of  cases,  the  ophthalmoscopic  picture  of  severe  hemorrhagic 
retinitis,  already  described,  is  due  either  to  thrombosis  or  obliterative 
phlebitis  of  the  central  vein,  or  both  combined,  very  rarelv  to  pressure  on 
the  vein  (Yamaguci).  Other  causes  which  have  been  advanced  but  not 
universally  accepted  are:  hemorrhagic  infarct  from  closure  of  the  central 
artery;  multiple  emboli  or  thrombi  in  the  retinal  veins  or  arteries;  de- 
generative changes  in  the  retinal  vessels  and  hemorrhage  into  the  substance 
of  the  optic  nerve. 

In  order  properly  to  estimate  the  value  of  the  pathological  findings 
in  a  given  case,  knowletige  of  the  methods  employed  in  making  the  exami- 
nation is  necessary.  Formerly,  it  was  the  practice  to  section  the  globe  and 
optic  nerve  longitudinally  or  to  cut  oft  the  nerve  close  to  the  globe  and 
section  it  transversely.  In  1900,  Haab  pointed  out  that  both  these  methods 
were  defective,  inasmuch  as  in  longitudinal  sections  an  obstruction  in  a 
vessel  is  exceedingly  apt  to  be  o\'erlooked,  while  in  nerves  cut  off  from 
the  eyeball,  the  usual  site  of  a  thrombus  in  the  lamina  cribrosa  was  not 
removed  and  examined  w^th  the  nerve.  By  the  present  method  trans- 
verse sections  of  the  ner^'e  and  posterior  segment  of  the  eye  are  so  made 
as  to  include  the  lamina  and,  furthermore,  everv  section  is  microscopically 
examined,  otherwise  failure  to  discover  an  obstruction  in  a  central  vessel 


144  THE    FUNDUS    OCULI. 

is  not  conclusive  evidence  that  it  does  not  exist.  Michel  goes  further  and 
does  not  consider  that  closure  of  the  central  vein  can  be  excluded  unless 
the  posterior  ends  of  the  central  vessels  are  examined,  especially  the 
sharp  bend  made  by  them  before'  passing  out  of  the  nerve  and  at  their 
passage  through  the  nerve  sheaths,  two  points  which  anatomically  favor 
thrombus  formation. 

Coats  says  the  vein  has  been  found  closed  at  some  point  in  every 
case  presenting  the  ophthalmoscopic  picture  of  thrombosis  in  which  the 
optic  nerve  and  lamina  cribrosa  have  been  sectioned  transversely  and 
every  section  examined.*  Harms  examined  10  cases.  In  1  the  lumen 
of  the  vein  was  obliterated  by  endo — and  mesophlebitis,  and  in  6  by  an 
organized  thrombus. t  While  most  authorities  hold  that  thrombosis  is 
the  usual  cause  of  central  vein  closure,  contrary  views  have  been  expressed. 
Thus,  Reimer  finds  but  three  cases  in  ophthalmologic  literature  which  he 
admits  to  be  true  thrombosis.  Other  cases  reported  as  such  he  regards 
as  phlebitis  or  endarteritis  proliferans.  Verhoff  believes  that  most  cases 
of  central  vein  obstruction  are  due  to  primary  endovas'^ular  proliferation. 

Can  the  ophthalmoscopic  picture  of  retinal  thrombosis  be  caused  by 
closure  of  the  central  artery  alone?  1  his  conception  is  founded  on  the 
well  known  phenomenon  of  hemorrhagic  infarction,  in  which  the  anemic 
area  produced  bv  embolism  of  an  artery  becomes  filled  with  extravasated 
blood.  The  source  of  the  blood  which  fills  an  infarct  has  been  the  subject 
of  extensive  speculation  and  research.  Virchow  suggested  all  the  theories 
which  have  since  prevailed  but  did  not  adopt  any  of  them.  In  1860 
Cohn  proved  that  hemorrhagic  infarct  was  not  due  to  reflux  of  blood 
from  the  veins  into  the  anemic  area.|  Litten,  repeating  the  experiments 
of  Cohn,  reached  a  similar  conclusion,  ?'.  e.,  that  the  blood  in  hemorrhagic 
infarction  does  not  come  from  the  veins,  but  from  the  capillaries  and 
small  collaterals  of  the  surrounding  vascular  tissue;  that  hemorrhagic  in- 


*He  microscopically  examined  16  cases.  In  14  the  vein  was  closed  by  an  organ- 
ized thrombus;    in  1   by  endophlebitis.  and  in   1   the  findings  were  inconclusive. 

t  Of  the  thrombus  cases  two  were  associated  with  advanced  disease  of  the  central 
artery,  three  with  disease  of  the  vein  wall  at  the  site  of  thrombosis  and  in  one  the 
thrombus  formed  in  an  apparently  normal  vein.  In  three  cases  the  morbid  anatomy 
was   not   determined   owing  to   imperfect   methods   of   examination. 

t  The  following  experiments  which  have  been  successfully  repeated  by  many  in- 
vestigators, prove  that  blood  does  not  enter  an  infarct  by  backflow  from  the  veins: 
1.  In  a  region  where  tying  an  artery  always  causes  hemorrhage,  if  both  artery  and 
veins  are  tied,  so  that  no  backflow  can  take  place,  bleeding  still  occurs,  in  fact,  it  is 
greatly  increased.  2.  In  a  region  where  tying  an  artery  is  not  followed  by  hemor- 
rhage, if  both  artery  and  veins  are  tied,  bleeding  occurs.  3.  If  all  direct  and  collateral 
vessels  to  a  region  are  tied  except  the  veins,  necrosis  ensues  but  no  hemorrha.ge. 


CLOSURE    OF    THE    RETINAL    VESSELS.  145 

farction  does  not  take  place  in  a  true  terminal  vascular  system  and, 
"  therefore,  cannot  occur  in  the  retina."  These  views  prevail  at  the  present 
time.  Notwithstanding  the  experiments  of  Cohn  and  Litten,  the  theory 
of  regurgitant  flow  from  the  veins  was  adopted  by  Conheim  in  1878, 
a  false  doctrine,  which  thus  acquired  an  impetus  that  has  scarcely  sub- 
sided to  this  day.  Naturally,  the  theory  of  hemorrhagic  infarction  has 
been  applied  to  cases  of  severe  retinal  bleeding  in  which  no  obstruction  was 
discovered  in  the  central  vein.  Opposed  to  this  view  are  such  authorities  as 
Haab,  Michel,  Coats  and  Harms,  who  are  convinced  that  hemorrhagic 
retinitis  does  not  result  from  obstruction  of  the  central  artery.  They 
admit  that  in  these  cases  the  artery  may  be  obstructed  but  insist  that  the 
vein  also  will  be  found  closed  if  the  specimen  is  examined  with  sufficient 
care.  It  is  a  matter  of  common  observation  that  closure  of  either  the 
central  artery  or  its  branches  is  not  usually  followed  by  severe  hemorrhage. 
Haab  cites,  from  21  authors,  39  cases  of  arterial  branch  closure  in  which 
no  trace  of  bleeding  occurred,  while  onlv  two  authors  were  found  who  saw 
faint  traces  of  hemorrhage.  On  the  other  hand,  in  the  cases  microscopically 
examined  by  Wagenmann,  Stolting,  Knall,  Meyerhoff,  Reimer  and  Oat- 
man,  hemorrhagic  retinitis  was  attributed  to  obstruction  on  the  arterial  side 
of  the  circulation.  The  majority  of  these  cases  were  sectioned  by  the  older 
methods  and,  therefore,  the  findings  cannot  be  considered  as  final.  This 
criticism,  however,  cannot  be  applied  to  either  Reimer's  or  my  own  case 
(page  112-113)  of  hemorrhagic  retinitis  in  which  no  obstruction  was  found 
in  the  central  ^'ein  while,  in  both  cases,  nearly  complete  closure  existed  in 
the  central  arterv.  Reimer  thinks  that,  in  his  case,  the  course  of  events 
was  temporary  closure  of  the  central  artery  followed  by  restoration  of 
circulation,  at  which  time  the  retinal  branches  gave  way  and  hemorrhage 
occurred.  This  theory  would  be  better  sustained  if  there  was  any  clinical 
or  microscopic  evidence  that  a  period  of  retinal  ischemia  preceded  the 
bleeding.  The  mechanism  of  the  hemorrhage  in  these  cases  is  best  ex- 
plained by  the  theorv  of  hemorrhagic  infarction,  which  Reimer  avoids. 
In  none  of  the  discussions  upon  retinal  hemorrhage  ha\'e  I  seen  any 
reference  to  the  important  experiments  of  Wm.  H.  Welch,  which  con- 
clusively demonstrate  that  hemorrhagic  infarction  mav  occur  in  a  terminal 
vascular  system,  like  the  retinal,  :and  that  the  blood  does  not  neces- 
sarily come  either  from  collateral  or  venous  sources.  The  experiments 
were  made  on  dogs.  All  vessels  communicating  with  the  intestine  were 
ligated  except  the  main  artery  and  vein.  A  loop  of  intestine  was  then 
tied  above  and  below  so  as  to  shut  oft  all  possible  sources  of  collateral 

10 


146  THE    FUNDUS    OCULI. 

blood.  Thus,  a  terminal  vascular  system  was  artificially  created.  So  far, 
no  bleeding  occurred.  Now,  the  artery  was  gradually  constricted  and  re- 
peated measurements  showed  that  hemorrhagic  infarction  invariably  oc- 
curred when  the  blood  pressure  was  thus  reduced  to  i/4  or  Vs  of  normal. 
If  the  artery  was  further  constricted  and  blood  pressure  fell  below  this 
point,  no  hemorrhage  took  place.  It  was  noticed  that  bleeding  always 
occurred  just  when  gradual  compression  arrested  lateral  pulsation  (pulse 
wave)  in  the  artery.  During  these  experiments,  at  no  time,  was  the  cir- 
culation entirely  arrested.  Welch  explains  the  hemorrhage  which  fol- 
lowed incomplete  closure  of  the  artery  as  follows.  The  escape  of  blood* 
when  pressure  falls  to  about  ^/4  of  normal,  depends  upon  certain  physio- 
logic properties  of  blooci  corpuscles,  principally  viscosity,  by  which  they 
stick  to  the  walls  of  the  vessel  when  not  dislodged  by  the  normal  pulse 
wave,  and  thus  time  is  given  them  to  squeeze  out  between  the  endothelial 
cells.  They  pass  out  of  the  vessel  entirely  by  diapedesis  and,  as  diapedesfs 
requires  a  certain  amount  of  pressure,  no  blood  escaped  when  the  blood 
pressure  was  reduced  below  a  certain  minimum.  These  experimentj 
indicate  the  possibility  of  hemorrhagic  infarction  resulting  from  incom- 
plete closure  of  the  central  artery.  Undoubtedly,  intraocular  pressure 
tends  to  prevent  hemorrhagic  infarction  in  the  retina.  It  usually  occurs 
where  tissue  resistance  is  low,  as  in  the  spongy  lungs  or  soft  mucosa  and 
sub-mucosa  of  the  intestines.  Further  experiments  by  Welch  were  as 
follows.  Rapid,  complete  closure  of  the  artery  with  the  vein  open  was 
followed  by  ischemia  but  no  bleeding,  which  corresponds  with  clinical  ex- 
perience in  closure  of  the  central  artery.  With  the  artery  open,  ligature  of 
the  vein  was  followed  by  intense  congestion  and  hemorrhage,  as  occurs 
in  thrombosis  of  the  central  vein. 

The  following  explanations  have  been  o<iered  to  account  for  general 
hemorrhagic  retinitis  in  special  cases.  Harms  is  disposed  to  accept  mul- 
tiple thrombi  in  the  retinal  veins,  without  thrombosis  of  the  central  vein, 
as  causative  for  two  of  his  doubtful  cases.  Coats  admits  the  possibilitv 
of  such  an  event  but  doubts  its  occurrence.  Alt  and  Ischeret  each  found 
thrombi  In  the  retinal  veins  but  no  closure  of  the  central  veins.  Both 
Wagenmann  and  Stoking  found  multiple  thrombi  in  the  retinal  arteries 
with  a  free  lumen  in  the  central  -vein.  Deutschmann  diagnosticated, 
"  closure  of  the  central  vein  from  hemorrhage  Into  the  substance  of 
the  optic  nerve,"   an  opinion  generally  regarded  as  erroneous. 

The  central  vein  like  the  artery  usually  is  obstructed  in,  or  close 
behind    the    rigid    lamina    cribrosa.      Occasionally    obstructions    form    »r! 


CLOSURE    OF    THE    RETINAL    VESSELS.  147 

Other  parts  of  its  course.  Thrombi  have  been  found  by  Alt  and  Wehrli 
anterior  to  the  disc.  In  one  case  by  Michel,  the  thrombus  extended  from 
the  papilla  to  the  exit  of  the  vein  from  the  nerve  and  in  another  it  was 
found  6  mm.  behind  the  lamina.  Yamaguci  found  a  thrombus  where  the 
vein  was  compressed  as  it  passed  out  through  a  greatly  thickened  nerve 
sheath. 

Authors  agree  upon  the  histologic  structure  oi  obstructions  found  in 
the  central  vein,  but  differ  as  to  their  interpretation.  The  chief  difficulty 
is  to  differentiate  an  organized  thrombus  from  endophlebitis.  This  arises 
from  the  fact  that  after  a  vein  has  been  closed,  either  by  primary  wall 
disease,  thrombosis  or  both,  secondary  changes  ensue  which  in  a  short 
time  present  the  same  microscopic  picture,  whichever  may  have  been  the 
original  cause  of  occlusion. 

Thrombosis.  A  thrombus  may  form  in  the  central  vein  as  a  result 
of  local  inflammation,  manifested  microscopically  by  dense,  round  celled 
infiltration  of  the  vessel  walls.  Such  cases  are  rare  and  generally  occur  in 
the  young,  especially  in  those  affected  with  gout,  rheumatism,  influenza 
and,  probably,  in  menstrual  suppression.  In  the  most  common  form, 
which  occurs  in  the  aged,  there  may  be  proliferation  of  endothelium  but 
no  inflammatory  thickening  of  the  walls.  Thrombus  formation  in  these 
cases  is  ascribed  to  changes  in  the  endothelial  cells  and  slowing  of  the 
blood  current,  perhaps  from  obstructive  disease  in  the  central  artery. 
Slowing  of  the  current  alone  is  insufficient  to  produce  thrombosis  as  shown 
by  the  following  experiment.  If,  in  a  living  body,  two  ligatures  are  carefully 
applied  to  a  blood  vessel,  so  as  not  to  injure  the  inner  coats,  the  enclosed 
blood  remains  fluid  for  weeks  (Glennard,  Baumgarten).  Von  Reckling- 
hausen regards  slowing  of  the  current  less  productive  of  thrombus  than 
whirling  and  eddying  of  the  blood  as  occurs  where  a  vein  Is  constricted  or 
bent.  The  so-called  marantic  thrombus  does  not  result  from  increased  co- 
agulability of  the  blood,  but  from  lesions  in  the  lining  of  the  vein  due  to  in- 
fective phlebitis.  Wright,  by  employing  selective  stains,  usually  has  been 
able  to  demonstrate  bacteria  in  marantic  thrombi. 

Thrombi  form  in  the  central  vein  by  the  same  processes  as  else- 
where.*     Coincident  with  the  formation  of  a  thrombus,  the  mass  is  in- 


*(!)  IVliife  thrombus — deposited  by  the  circulating  blood  and  consisting  of  blood 
platelets  and  fibrin.  The  deposit  is  quickly  invaded  by  leucocytes.  (2)  Red  thro)nhus,  or 
blood  clot,  which  represents  blood  suddenly  arrested  and  coagulated  en  masse.  This  variety 
consists  of  red  and  white  corpuscles  and  fibrillated  fibrin.  The  white  corpuscles  may  be  in 
excess.  (3)  Mixed  thrombus,  which  results  from  the  deposit  of  white  material,  by  circu- 
lating   blood,    upon    the    exposed    surface    of    an    incomplete    red    thrombus.       (4)   Hyaline 


148  THE    FUNDUS    OCULI. 

vaded  by  phagocytes  which  proceed  to  remove  the  blood  elements.  In 
large  vessels  the  further  processes  of  organization  are  carried  on  largely 
by  the  media,  adventitia  and  especially  the  vasa  vasorum,  but  in  small 
vessels  the  size  of  the  central  vein,  the  early  stages  are  carried  on  almost 
entirely  bv  the  endothelial  cells  of  the  vein,  which  proliferate  and  replace 
the  thrombus.  Connective  tissue  cells  push  in  from  the  walls  and  sur- 
rounding tissue  and  form  fibrous  septa.  Spaces  may  form  in  the  thrombus, 
become  lined  with  endothelium  and  filled  with  blood  by  new  vessels  which 
enter  from  the  vnsa  vasorum  (Figs.  97-98).  From  this  point  the  process 
of  organization  may  pursue  either  of  two  courses.  Tn  one,  the  new  formed 
blood  channels  may  enlarge  and  communication  be  re-established  through 
the  occluded  portion  of  the  vein.  This  is  called  canalization  of  a  thrombus 
and  has  been  observed  in  the  central  vein  by  Coats,  Harms,  Sider — Hu- 
gunin  and  Verhoff*  (Fig.  97).  Canalization  of  a  thrombus  is  exceptional. 
In  the  other  course,  the  cellular  deposit  is  replaced  by  connective  tissue 
and  the  vein  is  gradually  converted  into  a  fibrous  cord,  which  in  course 
of  time,  becomes  indistinguishable  from  the  surrounding  connective  tissue 
(Fig.  92).  Under  ordinary  conditions  these  processes  should  be  well 
started  within  a  week,  but  may  be  delayed  by  the  presence  of  pyogenic 
bacteria,  or  the  walls  of  the  vein  may  be  so  degenerated  that  they  cannot 
furnish  new  tissue.  After  a  thrombus  has  been  replaced  by  endothelial 
cells  or  fibrous  connective  tissue,  it  is  often  difficult,  if  not  impossible,  to 
determine  whether  the  primary  obstruction  was  a  thrombus  or  the  prod- 
ucts of  endovascular  proliferation.  In  either  case  the  lumen  finally  is 
closed  by  proliferative  vasculitis.  The  appearance  of  the  vessel  in  front 
of,  and  behind  the  point  of  closure,  may  assist  in  diagnosticating  the 
nature  of  the  obstruction.  Thus,  a  thrombus  terminates  at  the  point  where 
the  first  large  branch  enters  the  veint   (Fig.  96). 

Certain  peculiarities  in  the  anatomy  of  the  central  retinal  vein  should 
be  described.     In  the  optic  nerve  the  central  artery  consists  of  three  layers, 


tliroiiibiis.  This  variety  occurs  in  toxic  and  infections  conditions.  It  is  produced  by  ag- 
glutination (not  coagulation)  of  red  corpuscles  which  undergo  hemolysis  and  form  a  trans- 
lucent material  which  takes  Weigert's  fibrin  stain.  These  distinctions  are  recognizable  only 
ill  thrombi  of  recent  formation,  owing  to  changes  produced  by  the  processes  of  organiza- 
tion  which   immediately   ensue. 

*Verhoff   regards  the  process   of  canalization   as  a   form   of  dissecting  aneurism. 

t  Harms  differentiates  venous  obstructions  as  follows.  An  embolus  always  excites 
extensive  proliferation  of  the  intima  both  in  front  and  behind  the  point  of  lodgment. 
In  tlirninbosis.  on  the  proximal  side,  the  lumen  is  narrowed  from  infiltration  and  thickening 
of  the  walls  but  there  is  no  proliferation  of  the  intima.  On  the  distal  side  slight  intimal 
proliferation  sometimes  occurs.  Primary  phlebosclerosis  is  diagnosticated  when  high  grade 
inflammatory  thickening  of  the  wall  exists  on  both  sides  of  the  obstruction. 


Fig.  96 — A  large  Collateral  Branch 
joining  the  Central  Vein  at  the  point 
where    a    Thrombus    terminated. 


Fig.  90. 


Fig.  UT — Canalization  of  a  Thrombus, 
duo  to  the  Formation  of  Coalescing 
Spaces  which  become  Lined  with  Endo- 
thelium. Walls  of  Vein  Infiltrated  with 
Connective  Tissue. 


I'ij,'-  'Ji 


Fig.  98 — Another  instance  of  so- 
called  Canalization.  In  both  Figs. 
97  and  98,  the  Vasa  Vasorum  and 
Venules  of  the  Optic  Nerve  are  En- 
larged. 


Fig.  99. 

Central  Vein   Closed  by  Phlebitis  and  Thrombosis. 


Fig.  100. 

Sclerosis   of   Central  Vein. 

Walls  thickened  by  proliferation  of  cells  resembling  the  endothelial. 

(Section  3  m.  m.  behind  globe.) 


CLOSURE    OF     THE    RETINAL    VESSELS.  149 

i.  e.,  adventitia,  media  and  intima.  Ihe  intima  of  the  artery  is  distinguished 
from  that  of  the  vein  by  the  presence  of  a  homogeneous  elastic  membrane, 
while  the  vein  consists  only  of  an  endothelial  lining  resting  upon  a  thin 
wall  of  fibrous  tissue.  Although  the  walls  of  the  vein  contain  no  elastic 
membrane,  they  do  contain  a  large  number  of  individual  elastic  fibers. 
In  phlebosclerosis,  proliferation  of  elastic  tissue  may  be  the  dominant 
factor  in  thickening  the  walls.  In  such  cases  the  vein  is  microscopically 
differentiated  from  the  artery  by  employing  a  selective  stain  for  elastic 
tissue  which  will  exhibit  the  crcnated  membrane  of  the  artery  as  a  ho- 
mogeneous structure  which  cannot  be  further  resolved  by  the  microscope, 
while  new-formed  elastic  tissue  in  a  vein  consists  of  individual  fibers. 

In  phlebosclerosis  of  the  central  vein,  the  walls  usually  are  densely 
infiltrated  with  cells.  Later,  the  infiltration  assumes  the  form  of  connective 
tissue  (Fig.  97).  In  other  cases  the  predominant  factor  is  an  exuberant 
proliferation  of  cells  resembling  the  endothelial  (Fig.  100).  These  proc- 
esses may  greatly  reduce  the  calibre  of  the  lumen  without  producing  oc- 
clusion. In  such  cases  the  vein  is  lined  bv  a  smooth,  well  preser\ed  laver 
of  endothelial  cells,  over  which  the  blood  glides  with  increased  velocity 
without  depositing  a  thrombus.  When,  however,  this  endothelial  lining 
degenerates  or  is  lost,  a  thrombus  forms  and  the  vein  is  closed  bv  the 
combined  process  of  phlebitis  and  thrombosis  (Fig.  99).  In  other  cases 
the  vein  may  be  completely  closed  by  cellular  proliferation  alone.  The 
latter  process,  undoubtedly,  is  responsible  for  closure  of  the  central  vein 
in  a  large  proportion  of  the  cases  reported  as  thrombosis.  The  following 
is  an  example  of  a  vein  closed  by  this  method.* 

W.  Z.  Male,  54  years  of  age.  Had  general  arteriosclerosis.  Left 
eye.  The  first  ophthalmoscopic  examination  revealed  retinal  hemorrhages 
and  neuritis.  Four  years  later  he  returned  to  the  clinic  with  intravitreous 
hemorrhage  and  high  intraocular  tension.  Recurrent  bleeding  from  the 
iris  and  deeper  parts  contmued  for  the  next  six  weeks  when  the  eye  was 
enucleated.  The  most  interesting  pathological  feature  was  found  in  the 
condition  of  the  central  vein.  Commencing  about  3  mm.  behind  the  globe, 
the  walls  of  the  vein  were  infiltrated  with  epitheloid  and  round  cells  which 
thickened  the  walls  at  the  expense  of  the  lumen  (Fig.  100).  Infiltration 
and  proliferation  increased  towards  the  eye,  gradually  encroaching  upon 
the  lumen  until,  in  the  lamina  cribrosa,  the  lumen  was  obliterated  and  the 
vein  replaced  by  cells  and  organizing  connective  tissue    (Fig.   101).     No 


^From  the  Brooklyn  Eye  and  Ear  Hospital.     Service  of  Dr.  Henry  H.  W'augh. 


150  THE    FUNDUS    OCULI. 

blood  corpuscles  or  capillaries  existed  in  the  occluding  mass.  The  in- 
terpretation of  these  findings  was,  endophlebitis  proliferans.  The  process 
commenced  in  the  intima,  the  infiltration  extending  thence  to  the  walls 
(periphlebitis) . 

Clinical  History.  Contrary  to  general  experience,  Haab,  in  cases 
of  central  vein  closure,  has  met  with  prodromata  in  the  form  of  phosphenes 
and  temporary  obscurations  of  vision.  Where  such  a  history  exists,  disease 
of  both  central  vessels,  or  increased  intraocular  tension  should  be  suspected. 
Almost  invariably,  one  eye  only  is  affected,  which  illustrates  the  local 
character  of  vascidar  disease.  The  few  bilateral  cases  which  have  been 
reported  arose  in  the  course  of  acute  conditions  as  in  Cioh's  case  of  sepsis 
and  Gronow's  case  in  which  closure  of  both  central  veins  was  preceded 
by  three  days  of  unconsciousness.  In  fulminant  cases  vision  may  suddenly 
be  abolished  or  reduced  to  perception  of  light.  In  the  majority  of  cases, 
however,  vision  disappears  in  a  spasmodic  manner,  corresponding  to  suc- 
cessive hemorrhages.  Michel  observed  that  attacks  frequently  appear 
on  awakening  in  the  morning,  in  the  form  of  clouds  and  fog  before  the 
eye.  In  such  cases  it  may  be  assumed  that  local  conditions  favor  thrombus 
formation,  which  occurs  during  sleep  when  the  circulation  is  slow  and  the 
blood  pressure  low.  It  has  been  stated  that  rapid  onset  and  absence  of 
prodromata  point  to  closure  of  the  veins  by  thrombosis  rather  than  en- 
dophlebitis. Location  of  hemorrhage  has  an  important  influence  on  vision. 
Thus,  in  cases  of  moderate  severity  vision  will  be  very  low  if  bleeding  has 
occurred  in  the  macula,  while  in  severe  cases  considerable  vision  may  be 
present  if  the  niacula  has  escaped.  Cases  occurring  in  the  course  of 
retinal  arteriosclerosis  may  show  temporary  improvement  in  vision  from 
absorption  of  hemorrhages,  but  frequently  bleeding  recurs,  the  vitreous 
becomes  hazy  anci  total  lilindness  ensues.  Sometimes  violent  glaucoma  de- 
velops and  the  eye  is  enucleated  for  relief  of  pain.  In  incomplete  closure 
a  certain  amount  of  ^Msion  may  be  permanently  recovered.  In  very  rare 
cases,  usually  in  the  young,  vision  may  be  nearly  or  completely  restored 
after  closure  of  the  central  vein.  After  recovery  some  evidence  of  previous 
disease  remains,  such  as  slight  pallor  of  the  disc,  tortuosity  of  veins,  new 
formed  vessels  or  delicate  membranes  (Stergm.  49).  In  the  majority 
of  cases,  prognosis  is  bad  for  the  affected  eye.  As  to  the  fellow  eye, 
almost  positive  assurance  can  be  given  that  it  will  not  suffer  from  the 
same  disease.  The  presence  of  white  areas  in  the  retina  indicates  profound 
nutritive  disturbance  and  they  are  of  serious  import  as  regards  vision.  The 
after-history   of   retinal   thrombosis   has   not   been   traced   in   a   sufficient 


CLOSURE    OF     THE    RETINAL    VESSELS.  151 

number  of  cases  to  assist  in  estimating  the  subsequent  duration  of  life. 
Such  prognosis  must  depend  upon  the  systemic  conditions  which  caused  clo- 
sure of  the  vein.  Two  of  Coats'  cases  developed  symptoms  of 
cerebral  thrombosis.  He  watched  Hve  other  cases  for  periods  vary- 
ing from  one-and-a-quarter  to  five  years,  all  of  whom  remained  in  good 
health.  Three  cases  which  I  have  followed  for  seven,  five  and  three  years 
respectively,  are  all  in  good  physical  condition.  In  the  retinal  thrombosis 
affecting  young  women  with  irregular  menses  the  prognosis  appears 
good  as  regards  both  vision  and  duration  of  life. 

COMPLICA IIONS.  Closure  of  the  central  vein  not  infrequently  is 
followed  by  inflammatory  glaucoma.  The  percentage  of  cases  in  which  this 
occurs  has  not  been  ascertained.  Its  frequency,  however,  may  be  in- 
ferred from  the  fact  that  Coats  was  able  to  collect  16  cases  within  a  period 
of  four  years.  Wilbrand  and  Saenger  give  the  exact  time  which  elapsed 
between  closure  of  the  vein  and  onset  cf  glaucoma  in  40  cases.  Om'tt'n^ 
one  case  in  which  ten  years  elapsed,  the  average  time  was  14  weeks, 
varying  from  two  days  to  fifty-two  weeks.  I  have  observed  one  case  of 
retinitis   hemorrhagica    followed   by   iritis  without   glaucoma. 

Cases  of  thrombosis  which  first  come  under  ob3er\'at!on  in  a  glau- 
comatous condition,  usually  escape  detection  from  inability  to  elicit  a 
reliable  historv.  The  foIloM'ing  is  of  diagnostic  value  in  determining  the 
sequence  of  events.  Primary  thrombosis  of  the  central  vein  is  nearly 
always  unilateral,  while  primary  glaucoma,  nearly  always,  is  a  bilateral 
disease.*  If  hemorrhagic  retinitis  was  preceded  bv  the  prodromata  of 
glaucoma,  namelv,  temporarv  obscurations  cf  \ision,  iridescent  halo 
around  artificial  lights,  premature  presbyopia,  periorbital  pain  with  red- 
ness of  the  eve,  and  esoecially  if  svmntoms  of  glaucoma  exist  in  the 
other  eye,  It  may  be  assumed  that  glaucoma  antedated  the  hemorrhages. 
On  the  other  hand,  if  all  these  s^'mptoms  were  absent,  and  if  the  glaucoma 
is  limited  to  the  hemorrhagic  eve  and,  furthermore,  if  loss  of  vision 
was  rapid  and  appeared  first  on  waking  from  sleep,  the  case  may  be  con- 
sidered one  of  primarv  thrombosis  of  the  central  vein  with  secondary 
glaucoma.  The  expulsive  hemorrhage  which  occurs  in  glaucoma  after 
operations,  or  e\'en  spontaneously,  is  subretinal  and  supposed  to  come 
from  the  choroid.  The  depth  of  the  anterior  chamber  is  of  diagnostic 
importance.     Coats  has  brought  out  the  important  point  that  in  glaucoma 


*  V.  Graefe  regarded  the  association  of  r^tir?]  henT^rrh-ipf^s  and  .elaiicrr''  "s  *oo  fre- 
quent to  be  merely  coincidental.  His  observations  were  made  in  1869,  before  thrombosis  of 
the  central  vein  was  described  and  separated  from  glaucoma. 


152  THE    FUNDUS    OCULI. 

following  closure  of  the  central  vein,  the  anterior  chamber  is  not,  as  a 
rule,  shallow.  In  12  cases  which  he  microscopically  examined  and  noted 
the  depth  of  the  chamber,  it  was  normal  or  increased  in  11,  and  rather 
shallow  in  one.  Therefore,  in  a  given  case  of  monolateral  glaucoma,  in 
which  loss  of  vision  antedated  pain  in  the  eye  and  the  fundus  cannot  be  seen, 
if  the  anterior  chamber  is  very  shallow,  an  intraocular  growth  is  suspected. 
If,  however,  the  chamber  is  of  normal  or  Increased  depth,  this  is  evidence  In 
favor  of  primary  thrombosis  of  the  central  vein.  A  deep  anterior  chamber 
Is  of  more  diagnostic  value  than  a  shallow  one,  inasmuch  as  in  all  cases 
of  long  standing  glaucoma,  subject  to  recurring  inflammatory  attacks,  the 
area  of  adhesion  between  the  Iris  and  cornea  will  gradually  increase  until 
the  anterior  chamber  is  obliterated,  as  In  the  following  case.* 

Male,  54,  years  of  age.  The  right  eye  had  been  in  a  condition  of 
Inflammatory  glaucoma  for  several  months.  There  was  no  perception  of 
light.  The  loss  of  vision  preceded  pain.  The  anterior  chamber  was 
shallow.  \o  view  of  the  fundus  could  be  obtained.  Left  eye,  normal. 
Evidently,  this  was  a  case  of  glaucoma  secondary,  either  to  thrombosis 
or  tumor.  Considering  the  duration  of  the  Inflammatory  glaucoma,  the 
shallow  chamber  was  not  of  diagnostic  importance,  but  pointed  to  intra- 
ocular growth.  The  eye  was  enucleated  and  examined.  No  tumor  was 
found.  The  optic  nerve  and  posterior  segment  of  the  eye  were  sectioned 
transverselv  and  every  section  examined.  In  the  posterior  part  of  the 
lamina,  the  central  vein  was  occluded  by  a  well  organized  thrombus. 
Behind  the  thrombus,  for  a  distance  of  4  mm.,  the  vein  was  filled  with  a 
ciisorganized  blood  clot,  which  terminated  at  a  point  where  a  large  col- 
lateral joined  the  central  vein  (Fig.  96).  The  diagnosis  was,  glaucoma  sec- 
ondary to  thrombosis  of  the  central  vein. 

The  mechanism  by  which  closure  of  the  central  vein  and  retinal 
hemorrhage  causes  intraocular  tension  to  rise  has  not  been  positively  de- 
termined. Primary  glaucoma  does  not  appear  to  Induce  thrombosis  of 
the  central  vein.  When  these  conditions  coexist  I  have  found  wide  spread 
degeneration  of  all  the  retinal  vessels.  It  is  Inconceivable  that  interference 
with  the  lymph  channels  around  the  optic  nerve  could  cause  glaucoma. 
Inasmuch  as  no  amount  of  pressure  on  the  nerve  or  choking  of  the  disc 
influences  eye  tension,  so  long  as  anterior  drainage  Is  free.  Hemorrhage 
into  the  vitreous  is  uncommon  in  thrombosis.  It  occurred  in  none  of  the 
16  cases  examined  by  Coats.     Therefore,  the  glaucoma  is  not  due  to  ex- 


*Treate(l  liv  Dr.  G.  A.  Hare,  at  the  ]\Ianhatran  Eye,  Ear  and  Throat  Hospital. 


Fig.  101 — From  the  same  spec- 
imen as  Fig.  100,  but  section 
through  t'.U"  Lamina  Cribosa. 
The  Lumen  of  the  Vein  (to  the 
left")  is  Completely  Occluded 
and  the  Vein  Replaced  b>  Cells 
and  Connective  Tissue.         ' 


Fig.  101. 


Fig.  103  —  From  sL 
case  of  Glaucoma  sec- 
ondary to  Thrombosis 
of  the  Central  Vein. 
The  Anterior  Chamber 
is  deep  but  the  Pecti- 
nate is  thickened  and 
Fontana's  spaces  are 
filled  with  Pigm'-nt 
and    Cells. 


Fig.   102. 


CLOSURE    OF    JHE    RETINAL    VESSELS.  153 

travasated  blood  which  has  increased  the  volume  of  the  vitreous.  The 
lens  was  not  disproportionately  large  in  any  of  Coats'  cases.  In  several 
cases  of  thrombosis,  glaucoma  has  been  precipitated  by  the  use  of  a  myd- 
riatic, which  shows  the  glaucomatous  tendency  of  this  affection.* 

Angiosclerosis  alone  or  combined  with  thrombosis  is  the  dominant 
factor  in  closing  the  central  vein.  Sclerosis  of  the  channels  which  drain 
the  anterior  chamber,  also  appears  to  be  causative  in  certain  cases  of 
glaucoma.  Notwithstanding  this  apparent  relationship,  sound  objections 
exist  against  attributing  both  closure  of  the  vein  and  a  subsequent  glau- 
coma to  vascular  degeneration.  Primary  glaucoma,  whether  due  to  angio- 
sclerosis or  some  other  cause  is  nearly  always  bilateral,  while  glaucoma 
following  closure  of  the  central  vein  is  unilateral  and,  moreover,  alway3 
occurs  on  the  same  side  as  the  thrombosis.  Therefore,  the  deduction  ij 
that  post-thrombotic  glaucoma  is  secondary  to,  and  directly  dependent 
upon  closure  of  the  central  vein  and  hemorrhage.  The  glaucoma 
appears  to  depend  upon  the  amount  of  blood  extravasated.  Certain, 
fairly  constant  peculiarities  are  observed  in  the  anatomy  of  post-thrombotic 
glaucoma  which  distinguish  it  from  the  usual  chronic  type.  (n)  The 
anterior  chamber  is  not  shallow;  {b)  The  fibers  of  the  pectinate  ligament 
frequently  are  thickened  and  the  spaces  of  Fontana  filled  with  pigment 
and  endothelium  (Fig.  102);  {c)  A  sluggish,  localized  inflammatory 
process  exists  in  the  sinus  of  the  anterior  chamber,  manifested  by  slight 
peripheral  adhesions  between  the  iris  anci  cornea,  and  sometimes  by  cel- 
lular infiltration  of  the  tissues  around  Schlemm's  canal.  Recent  iritic 
adhesions  were  found  in  1  1  of  Coats'  cases.  It  is  remarkable  that  in  9 
of  these  the  adhesions  were  limited  to  the  temporal  side  of  the  chamber 
and,  when  present  on  both  sides,  were  much  narrow^er  on  the  nasal.  This 
differs  from  common  glaucoma  in  which  the  iris  is  uniformly  attached  all 
around. t  In  thrombotic  cases  increased  tension  is  not  due  to  slow  filtra- 
tion from  increase  in  the  albuminous  constituents  of  the  aqueous,  inasmuch 
as  the  aqueous  appears  to  be  less  albuminous  than  in  ordinary  iridocyclitis. 
The  most  reasonable  explanation  for  development  of  glaucoma  after 
intraocular  hemorrhage,  has  been  offered  by  Baques  who,  in  a  case  of 
retinal  bleeding  found  Fontana's  spaces  filled  with  leucocytes  and  pro- 
liferating endothelium.  There  was  also  cellular  infiltration  and  thicken- 
ing of  the  pectinate  ligament.    This  was  regarded  as  a  preglaucomatous  con- 


*  In  four  of  Coats'  cases,  glaucoma  followed  dilatation  of  the  pupil. 
tWith  the  possible  exception  of  certain  hemorrhagic  cases  of  glaucoma  in  which  bleed- 
ing occurred  early  in  the  disease. 


154  THE    FUNDUS    OCULI. 

dition,  caused  by  the  presence  in  the  aqueous  of  irritating  chemical  products 
from  decomposing  blood  and  dead  retinal  elements.  These  products  are 
supposed  to  produce  local  infection  and  inflammation  in  the  pectinate 
which  thickens  its  fibers  and  gradually  converts  its  open  meshwork 
into  solid  tissue.  7  o  this  should  be  adcied  blocking  of  the  filtration  spaces 
by  phagocytes  loaded  with  products  of  degeneration  from  the  hemor- 
rhagic areas.  This  theory  is  supported  by  Inouye,  who  calls  attention  to 
the  fact  that  toxins  from  extravasated  blood  are  hemotactic,  /".  e.,  they 
stimulate  the  tissues  to  furnish  material  for  cicatrization.  After  intra- 
ocular hemorrhage  the  pectinate  ligament  is  subjected  to  this  action  and 
responds  by  proliferation  of  tissue.  If  the  retinal  vessels  are  sclerosed, 
absorption  of  blood  will  be  delayed  and  the  action  of  the  blooci  toxins 
prolonged. 

The  following  case  which  came  under  my  observation  appears  to 
illustrate  Baques'  theory.  A  laboring  man,  58  years  of  age,  with  well  ad- 
vanced arteriosclerosis,  entered  my  clinic  complaining  of  blurred  vision  in 
the  left  eye.  Right  eye  presented  evidence  of  retinal  arteriosclerosis.  Oph- 
thalmoscopic examination  of  the  left  eye  revealed  the  picture  of  complete 
retinal  thrombosis.  Three  weeks  later  violent  glaucoma,  with  deep  an- 
terior chamber,  developed  and  the  eye  was  enucleated.  The  appearance 
of  the  filtration  angle  is  shown  in  figure  102.  Points  of  recent  adhesions 
between  the  root  of  the  iris  and  the  pectinate  existed,  especially  on  the  tem- 
poral side  of  the  eye.  The  adhesions  were  situated  at  a  slight  distance  from 
the  angle  of  the  chamber.  The  adhesions  were  readily  broken  as  has  oc- 
curred in  the  section  shown  in  the  cut.  The  strands  of  the  pectinate  were 
thickened  and  Fontana's  spaces  completely  filled  by  endothelial  cells, 
leucocytes  and  pigment.  Figure  103  shows  a  section  of  retina  destroyed 
by  hemorrhage  from  which  the  extravasated  blood  has  been  removed. 
Pigment-laden  leucocytes  are  seen  passing  out  into  the  vitreous. 

It  is  not  clear  why  the  temporal  side  of  the  anterior  chamber  should 
be  more  subject  to  inflammation  than  the  nasal,  unless,  as  appears  true, 
retinal  hemorrhage  is  always  more  extensive  in  the  temporal  half  and 
the  Irritating  products  from  decomposing  blood  are  discharged  chiefly 
through  this  side  of  the  chamber.  The  relative  frequency  with  which 
hemorrhage  occurs  on  the  two  sides  of  the  eye  is  shown  by  Ammann,  who 
collected  13  cases  of  branch  retinal  thrombosis,  all  of  which  occurred  on 
the  temporal  side. 

Iritis  is  an  occasional  sequel  to  retinitis  apoplectlca.  T  have  seen 
one   case   which    recovered    after    resorption    of   the    extravasated   blood. 


CLOSURE    OF    THE    RETINAL    VESSELS.  155 

Probably,  like  postthrombotic  glaucoma,  it  is  caused  by  the  local  action 
of  toxins  from  decomposing  blood. 

Branch  Closure.  Closure  of  a  branch  retinal  vein  produces  the 
picture  of  hemorrhagic  retinitis  limited  to  a  section  of  the  fundus,  drained 
by  the  occluded  vessel  (Fig.  104).  If  the  vein  is  closed  anterior  to  the 
disc,  the  latter  probably  will  be  free  trom  swelling.  Ammann  collected 
90  cases  of  retinal  hemorrhage  from  various  causes.  Of  these,  20  were 
due  to  closure  of  the  central  vein  and  13  to  closure  of  its  branches.  About 
two-thirds  of  the  branch  cases  occurred  in  the  superior  temporal  and 
one-third  in  the  inferior  temporal  branch.  Branch  closure  is  said  to 
occur  at  a  somewhat  earlier  age  than  closure  of  the  trunk  \essel.  Ar- 
teriosclerosis of  both  veins  and  arteries  can  frequently  be  seen.  Hemor- 
rhage due  to  closure  of  small  retinal  veins  occurs  in  diseases  associated 
with   \ascular   degeneration,    as   Bright's   disease,   diabetes,    etc. 

Sii'.REOGRAM  48.  Closure  of  Superior  Retinal  Vein  (7'hrom- 
bosis).  Right  eye  of  laborer,  49  years  of  age.  Blood  pressure  195  mm. 
Hg.  Chronic  interstitial  nephritis.  Left  eye  is  blind  from  old  detach- 
ment of  the  retina.  Five  days  ago  the  sight  in  the  right  eye  rapidly  failed. 
Vision  now  is  20/70.  The  ophthalmoscopic  picture  is  characteristic  of 
closure  of  the  ascending  branch  of  the  central  vein.  This  vessel  and  its 
branches  are  dilated,  tortuous  and  accompanied  by  hemorrhages.  The 
optic  disc  is  slightly  swollen  and  its  temporal  margin  is  obscured.  Through- 
out the  region  drained  by  the  affected  \ein,  the  retina  is  edematous  and 
opaque.  Spots  of  intense  edema  or  exudation  are  scattered  along  the 
veins,  particularly  where  they  make  a  quick  bend  and  dip  into  the  thickened 
retina.  Both  superficial  and  deep  extravasations  have  occurred.  A  small, 
round  hemorrhage,  situated  near  the  macula,  was  at  first  mistaken  for 
a  red  foveal  spot.  This  error  was  corrected  by  directing  the  patient  to 
gaze  into  the  aperture  of  the  ophthalmoscopic  mirror,  when  the  point  of 
fixation  (fovea)  was  found  to  be  above  and  nearer  the  disc  than  the 
round  hemorrhage.  The  veins  not  included  in  the  obstruction  are  some- 
what tortuous.  This  is  attributed  to  retinal  arteriosclerosis  which  is 
further  demonstrated  by  the  descending  arterv  which  indents  and  hides  a 
vein  over  which  it  crosses.  The  mild  neuritis  may  be  caused  by  associated 
albuminuria   or  by  the   situation   of  the   thrombus   on   the   disc. 

Diagnosis.  In  this  case,  the  diagnosis  of  venous  branch  closure  rests 
upon  the  presence  of  hemorrhage,  exudation,  tortuous  veins,  comparatively 
unaltered  arteries  and  retinal  edema,  limited  to  the  region  drained  by  a 
branch  vein.      Primary  optic  neuritis  is  excluded  on  the  grounds  that  so 


156  THE    FUNDUS    OCULI. 

much  edema  and  hemorrhage  could  not  be  caused  by  moderate  swelling  of 
the  nerve,  and  also  by  the  limited  area  of  retina  affected. 

Stereogram  49.  Postthrombotic  Changes  (New  Formed  \^eins 
AND  Membranes).  Left  eyeground  of  woman,  30  years  of  age. 
Two  years  ago  the  central  vein  in  this  eye  was  closed.  The  picture  of 
hemorrhagic  retinitis  existing  at  that  time  is  shown  in  stereogram  47. 
Vision  is  now  restored  to  20/30.  The  optic  disc  is  physiologically  ex- 
cavated and  the  cup  is  filled  with  new-formed  connective  tissue  which 
extends  bevond  the  nerve  in  the  form  of  membranes  covering  the 
retinal  vessels.  The  membrane  which  follows  the  descending  vessels  is 
diaphanous  and  covered  by  small  vessels,  while  the  ascending  vein  is  en- 
wrapped by  a  thick,  membranous  formation  which  thickens  the  vessel  but 
does  not  appear  to  interfere  with  circulation.  The  vascular  changes  ha\'e 
occurred  chieflv  on  the  temporal  side  of  the  nerve.  The  superior  temporal 
vein  is  obliterated  beyond  the  edge  of  the  disc.  Reference  to  stereogram 
47  will  show  that,  in  the  early  period  of  closure,  this  vessel  appeared  to 
terminate  in  a  deep,  round  hemorrhage.  The  macular  region  to  which 
this  vein  once  was  distributed,  is  now  drained  bv  a  large  number  of  newly 
developed  vessels  connected  with  the  stump  of  the  obliterated  vein.  The 
inacular  region  is  further  supplied  anti  almost  encircled  by  a  greatly  en- 
larged branch   from  the   ascending  vein. 

When,  in  its  passage  through  the  optic  nerve,  the  central  vein  is 
closed  by  a  thrombus,  circulation  is  re-established  by  enlargement  of  the 
vasa  vasorum  and  the  venules  in  the  nerve  (Fig.  98).  In  a  similir 
manner,  if  a  retinal  vein  is  permanently  obstructed,  the  small  tributaries 
become  greatlv  enlarged  and  in  course  of  time  an  efficient  collateral  flovr 
will  be  established  (Stergm.  30).  Another  occasional  sequel  to  retinal 
hemorrhage  is  the  formation  of  membranes,  fibrous  bands,  etc.  (Stergms. 
61-62).  Why  this  occurs  in  one  case  of  bleeding  and  not  in  another,  is 
unknown. 

Obstruction  of  both  Central  Vessels.  Clinically,  the  two  con- 
ditions, closure  of  the  central  artery  and  of  the  central  vein,  are  sharplv 
differentiated;  but  in  either  affection,  microscopical  examination  frequently 
reveals  more  or  less  obstructive  disease  in  both  vessels.  In  such  cases, 
however,  the  arterv  and  vein  appear  never  to  be  affected  by  the  same 
chronic  pathological  process,  /.  e.,  double  thrombosis  or  double  endovascu- 
litis.*     According  to  Harms  nearly  complete  closure  of  either  the  central 


*  Gonin's  case  of  doulile  thrombosis  from  external  pressure  on  both  vein  and  artery, 
was  a  mechanical,  not  a   disease  process. 


CLOSURK    OF     I'HK    KIllINAl.    \  IISSELS.  157 

vein  or  artery,  by  retarding  the  blood  stream,  excites  reciprocal  disease  in 
the  other  vessel.  In  a  measure  this  is  due  to  the  fact  that  the  retinal  cir- 
culation is  terminal,  and  carried  on  under  high  (intraocular)  pressure. 
Under  these  conditions,  if  circulation  is  obstructed  in  either  central  vessel 
or  in  the  smaller  retinal  branches,  the  entire  retinal  system  is  affected. 
Thus,  a  thrombus  in  the  central  vein,  by  damming  the  blood,  may  excite 
intima  proliferation  in  the  artery.  On  the  other  hand,  an  endarteritic 
nodule  in  the  central  artery  may  retard  the  blood  current  sufficiently  to 
induce  thrombus  formation  in  the  vein.  in  a  similar  manner  diffuse 
sclerosis  of  the  retinal  vessels  may  react  upon  one  or  both  of  the  central 
vessels;  in  fact,  arteriosclerosis  not  infrequently  appears  first  in  the  smaller 
branches.  I'he  lumen  of  a  central  artery,  or  vein  secondarily  affected 
may  close  first,  although  obstruction  to  circulation  began  in  the  other 
vessel.  For  example,  while  the  ophthalmoscopic  picture  of  either  "  em- 
bolism "  or  apoplexy  shows  in  which  central  vessel  the  disease  has  ter- 
minated, it  does  not  necessarily  indicate  the  one  in  which  it  originated. 
Neither  can  this  point  always  be  determined  by  microscopic  examination. 
The  following  is  an  illustrative  case.  The  patient  was  a  drunkard, 
45  years  of  age.  The  surface  arteries  were  highlv  sclerosed.  The  day 
before  entering  the  clinic,  vision  in  the  right  eve  rapidlv  decreased  to 
movements  of  the  hand.  Ophthalmoscopic  examination  showecl  the  retina 
covered  with  hemorrhages,  one  of  which  occupied  the  macula.  The  retinal 
veins  were  tortuous  and  dilated.  Arteriosclerotic  changes  existed  in  the 
retinal  arteries.  Four  months  later  an  extensive  hemorrhage  occurred 
into  the  vitreous.  Glaucoma  developed  and  the  eve  was  enucleated.  Mi- 
croscopic examination  revealed  closure  of  both  central  vessels  as  they 
passed  through  the  lamina  cribrosa,  the  artery  being  closed  by  endarteritis 
proliferans  and  the  vein  by  thrombosis.  Behind  the  lamina  the  vein  was 
almost  indistinguishable  from  the  perivascular  connective  tissue  (Fig.  88). 

Bibliof/rnphy. 

Amman,   Beilrag.   z.   prak.    Augen.   XXXVITI,  p.   1. 

Alt,  Am.  Jour.  Oph.  XV,  p.  298. 

Balaban.   A.   f.   A,  June   1900;     Trans.   A.   of   Oph.,   XXXTV,   190.5,  p.    177.   A.    f.    A. 

XLI,  280. 
Baum.oarten,   d.   sogan.    organiz.    d.   thromb.   Leipzig,   1877. 
Baques.   A.    f    O.,   LXVIII. 
Bondi,   Prag.   Med.  Wochens.,   1901.  XXVI. 

Briicke.  British  &  Foreign   Med.  &  Chirg.  Rev.,   18.57.  XIX,  p.   183. 
Clermont,   These  de   Paris,   1900. 

Coats,  O.  J^.  Rep.,  XIV,  1.  1904,  p.  516:    XVI,  4.  1906,  516;    T.  O.  Soc,  XIV,  1904,  110. 
Conheim,   Untersuch.  u.   d.  emb.  processe,   Berlin,  1872. 
Cohn,   Klin.  d.   Eml).  gefasskrankh.,   Berlin,   1860. 


158  THE    FUNDUS    OCULI. 

Deutschmcinn,  A.   f.  O.,  XXXV,  3.  1879. 

Glennard,  These  de  Paris,  1875. 

Goh,  A.  f.  C,  XLTIT,  1897,  p.  166. 

Goldzeiher.  C.   t.  p.  A.,  XXVIII.  1904,  p.  257. 

Gonin,  A.   d'Ottal.,   XXIII,   1903,  2,  219. 

Groenouw,  Graefe  u.  Saemisch,  II,  p.  54-55,  625. 

Haab,   Norris  &  Oliver,  IV,  p.   515  ;    28  Versamml.   z.   Heidelburg,  1890,  210. 

Harms,  Rep.  10th  Internal.  Cong.,  A.  of  Oph.  XXXIV,  1905,  p.  202.  A.  f.  O.   LXI,  1905. 

Hinschberg.   C.    f.    p.   A.,  X,   1886;     XV.    1891. 

Hormuth,  K.  m.   f.  A.   Beilag.     Festschrift  f,  Manz  u.   Sattler,   1903,  p.  255. 

Inoyu,   O.   H.  Rep.,  XVIII,   1910,-  24. 

Ischeret,  "A.   f.   A.,   1900,   38. 

Knapp,  A.  f.  A.,  1,  1869. 

Litten,   Uber   d.    Hemorrhag.    infark.    Berlin.    1879;     Zeits.    f.    Klin.    Med.    Ed.    1,   1880 

Arch.  f.  Oph.  LXIII. 
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Moses,  Inaug.  Dissert.,  Wiirtzburg.  1896. 

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of  Oph.  XXIX,  347. 
Schonewald,    Inang.   Dissert.,   Giessen,   1900,  p.   619. 
Sider-Hugunin,  A.   f.   A.,  LI,  1904. 
Stolting,  A.   f.   O.,  XLIII,   1897. 

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Waggenmann.  A.  f.  O..  XXXVIII.  3.  213. 
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p.  550. 
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Chapter  VI IT. 
DETACHMENT  OF  THE  RETINA. 

In  considering  the  subject  of  retinal  detachment,  the  layer  of  pig- 
mented epithelium  is  regarded  as  part  of  the  choroid  to  which  it  ana- 
tomically and  pathologically  belongs.  In  its  normal  position,  the  retina 
simply  lies  in  contact  with  the  choroid,  the  only  attachments  being  at 
the  posterior  pole  where  its  libers  unite  to  form  the  optic  nerve,  and  at  the 
anterior  border  where  it  merges  with  the  subjacent  parts  to  form  the 
ora  serrata.*  1  he  retina  is  held  in  apposition  with  the  choroid  by  the 
vitreous  body,  the  force  exerted  being  equal  to  that  of  the  intraocular  pres- 
sure. Separation  of  the  retina  is  ettected  in  various  ways.  Thus,  it  may  fall 
inwards  when  support  is  withdrawn,  as  after  extensive  loss  of  vitreous 
from  wounds  and  operations,  or  it  may  be  dragged  from  its  bed  by  con- 
traction of  new-formed  tissue  in  the  vitreous.  In  other  cases  the  retina 
may  be  pushed  away  from  the  choroid  by  subretinal  effusions,  displaced 
vitreous,  blood,  tumors,  cysticerci  and  penetrating  bodies.  Detachment 
often  follows  the  application  of  blunt  force  to  the  eye.  In  a  clinical  sense, 
"  detachment  of  the  retina  ''  refers  only  to  that  form  of  retinal  displace- 
ment in  which  a  translucent  fluid  accumulates  between  the  retina  and  cho- 
roid. This  is  known  as  serous  detachment  and  also,  in  ignorance  of  its 
etiology,  simple  detachment. 

Si:r()US  Detachment.  In  this  form  of  detachment  separation  of 
the  retina  usually  occurs  in  an  unaccountable  manner  or  from  slight  causes, 
as  stooping,  sneezing,  vomiting,  etc.  Hence,  it  is  sometimes  called  "  spon- 
taneous detachment."  As  a  rule,  when  it  first  comes  under  observation, 
detachment  is  extensive  and  the  retina  greatly  elevated.  Exceptionally,  the 
amount  of  subretinal  fluid  is  slight  and  the  surface  of  the  detachment  but 
little  higher  than  the  surrounding  fundus,  constituting  the  condition  known 
as  flat  detachment.  A  slight  detachment  of  the  retina  may  remain"  flat." 
Usually,  however,  it  increases  in  size.     As  a  rule,  it  is  due  to  injury,  espe- 


*  The  terms  "detachment"  and  "reattachment"  of  the  retina  are  somewhat  mislead- 
ing.    Strictly  speaking,  the  retina  is  not  detached,  but  is  displaced  or  dislocated. 

159 


160  THE    FUNDUS    OCULI. 

cially  in  myopic  eyes.     In  such  cases  a  slight  blow  may  be  followed  weeks 
later  by  detachment  which  at  first  is  flat. 

Stereogram  50.  Incipient  Detachment  of  the  Retina  (Flat 
Detachment) .  Left  eyeground  of  a  man,  35  years  of  age.  Two  weeks  ago, 
while  boxing,  he  received  a  stiff  blow  over  the  left  eye.  Ten  days  later,  pho- 
topsi^e  appeared  in  the  form  of  flery  sparks  and  flashes.  Vision  is  re- 
duced and  objects  appear  distorted.  The  eye  is  myopic  6.S  D.  All  parts 
of  the  fundus  present  a  red  reflex.  The  optic  disc  is  uniformly  reddened 
and  on  its  temporal  side  is  a  narrow,  myopic  crescent.  In  the  upper  tem- 
poral quadrant,  the  surface  of  the  retina  is  covered  by  a  number  of  light, 
parallel  lines  which  disappear  on  changing  the  position  of  the  eye.  In  this 
region  the  retinal  arteries  and  veins  are  darkened  and  contain  no  central 
light  streaks.  They  also  are  wavy  as  though  following  wrinkles  in  the 
retina.  It  cannot  be  determined  by  the  ophthalmoscope  that  the  surface 
is  elevated.  In  the  lower  half  of  the  fundus  the  retinal  vessels  are 
straightened  and  drawn  toward  the  temporal  side,  as  is  common  in  myopia. 

The  ophthalmoscopic  picture  in  this  case  is  that  of  incipient  detach- 
ment of  the  retina,  the  streaked  appearance  being  due  to  light  reflected 
from  inequalities  on  its  surface.  It  is  of  the  greatest  importance  to  recog- 
nize a  retinal  detachment  in  the  incipient  stage.  For  this  reason,  myopic 
eyes  which  have  been  injured  should  be  examined  frequently  for  early 
evidence  of  retinal  separation.  While  the  ophthalmoscopic  picture  of  high 
retinal  detachment  is  unmistakable,  that  of  flat  detachment  is  recognized 
with  difficultv,  in  fact,  it  frequently  is  overlooked. 

Diagnosis.  In  flat  detachment,  if  the  subretinal  fluid  is  clear  the 
normal  red  fundus  reflex  may  not  be  changed.  In  such  cases  the  diagnosis 
of  incipient  detachment  may  be  established  by  the  presence  of  some  or  all 
the  following  symptoms : 

(n)  Functional  disturbance  consisting  of  lowered  visual  acuity,  tor- 
por retinae,  metamorphopsise,  photopsiae,  chromotopsia?,  etc. 

(b)  Congestion  and  darkening  of  the  vessels  with  loss  of  central 
light  streak  and,  sometimes,  irregularities  in  their  course. 

(c)  Local  hypermetropia  demonstrable  by  the  ophthalmoscope. 

(d)  When  underlying  choroidal  vessels  are  visible,  a  parallactic  mo- 
tion between  them  and  the  retinal  vessels  may  be  obtained,  indicating  an 
abnormal  difference  in  levels. 

(e)  Wrinkles  in  the  retina,  demonstrable  either  by  surface  light  re- 
flexes or  by  the  course  of  retinal  vessels.  Not  all  flat  detachments  are 
obscure;  some  are  grey  and  easily  recognized. 


DETACHMENT    OF    THE    RETINA.  161 

Retinal  opacity  due  to  closure  of  a  branch  artery  may  be  mistaken 
for  flat  detachment.     Typical  cases  present  the  following  difterences: 

(  1 )  The  subjective  symptoms  of  detachment  are  absent  in  closure  of 
a  retinal  artery. 

(2)  In  flat  retinal  detachment,  a  fair  amount  of  vision  is  retained, 
while  the  area  to  which  an  occluded  artery  is  distributed  is,  or  has  been, 
blind. 

(3)  In  arterial  closure  the  surface  of  the  retina  is  not  elevated. 

(4)  In  detachment,  the  afiected  region  bears  no  relation  to  the  ves- 
sels, but  an  obstructed  artery  produces  disturbance  throughout  a  triangular 
area  corresponding  to  its  distribution. 

i^)  The  chief  distinction  is  the  appearance  of  the  retinal  vessels, 
which  in  closure  are  narrowed  or  obliterated,  while  in  detachment  they  are 
abnormally  full  and  dark. 

In  retinal  edema  due  to  choroidal  eflusion  (Stergm.  18)  the  retina 
may  be  separated  from  the  choroid.  This  condition  may  be  differentiated 
from  simple,  serous  detachment  by  the  retinal  edema  which  may  cover  the 
vessels,  and  by  the  presence  of  other  inflammatory  choroidal  lesions.  The 
possible  presence  of  a  young,  intraocular  tumor  must  be  borne  in  mind  when 
viewing  a  circumscribed  detachment  of  the  retina. 

Symptoms  of  Ri  tinal  Detachment.  The  great  majority  of  de- 
tached retinas  are  widely  separated  from  the  choroid  and  occupy  one-half, 
or  more,  of  the  fundus  (Pig.  105).  In  uncomplicated  serous  detachment 
the  eye  presents  no  external  signs  of  irritation.  The  pupil  is  moderately 
ciilated  and  reacts  sluggishly  to  direct  light.  As  a  rule,  the  lens  recedes 
and  the  anterior  chamber  deepens.  The  lens  may  fail  to  support  the  iris 
and  the  latter  trembles  when  the  eye  is  moved  (iridodonosis).  Ocular 
tension  is  normal  in  recent  detachment  but  in  old  detachment  it  may  be 
diminished.  It  is  never  increased,  although  in  rare  cases  it  may  be  raised 
by  coexisting  disease.*  In  high-grade  detachment  with  a  wide  pupil,  it 
may  be  possible  to  see  the  surface  of  the  retina  with  the  naked  eye.  With 
the  ophthalmoscope  no  difficulty  is  experienced  in  recognizing  a  developed 
detachment.  By  simple  illumination  of  the  fundus  a  portion  of  the  field 
will  be  observed  which  throws  a  light  colored  reflex.  Upon  detailed  ex- 
amination this  is  recognized  as  detachment  of  the  retina  by  its  height  and 


"*  Xordenson  examined  the  intraocular  tension  in  126  cases  of  detachment  and  in  only 
(i  was  it  increased.  Of  these,  1  had  occlusion  of  the  pupil,  3  had  iridocyclitis  and  in  2 
the  cause  was  not  apparent.  It  is  doubtful  if  detachment  occurs  in  glaucomatous  eyes, 
but  in  an  eye  with  detachment  established,  conditions  might  arise  in  the  anterior  chamber 
that  would  raise  the  tension. 
11 


162  THE    FUNDUS    OCULI. 

from  a  tremulous,  wave-like  motion  which  passes  over  its  surface  upon 
the  slightest  movement  of  the  eye.  When  first  separated  from  the  choroid 
the  retina  is  transparent  and  for  a  brief  time  presents  a  red  reflex,  but  it 
quickly  becomes  edematous  and  opaque.  Usually,  a  detached  retina  has  a 
grey  lustre  resembling  the  sheen  of  a  silky  fabric.  The  presence  of  a 
very  dark  green  cast,  indicates  that  blood  is  mixed  with  the  subretinal 
fluid.  Simple  detachment  seldom  forms  a  single  rounded  mass,  but  pre- 
sents a  number  of  bulging,  bladder-like  elevations  the  summits  of  which  re- 
flect light  with  a  whitish  sheen  (colored  plate  4).  In  other  cases  the 
surface  is  folded,  or  it  may  be  broken  into  numerous  wrinkles.  The 
border  of  a  detachment  may  be  baggy  and  overhanging,  or  it  may  merge 
into  the  fundus  with  no  distinct  line  of  demarcation.  Whitish  lines  or 
linear  folds  sometimes  are  seen  at  the  edge  of  a  detachment,  especially 
along  the  upper  margin  of  those  which  recently  have  changed  their  position. 
The  detached  retina  is  hyperopic.  This  fact  enables  the  observer  to  as- 
certain the  height  of  the  detachment,  by  measuring  with  the  ophthalmoscope 
the  difference  in  refraction  between  its  summit  and  the  normal  level  of  the 
fundus,  computing  3  dioptres  of  refraction  as  equal  to  1  mm.  in  height. 
The  appearance  of  the  blood  vessels  in  a  detached  retina  is  characteristic  of 
the  affection.  They  accurately  follow  the  folds  and  undulations  of  the 
retina,  riding  prominently  over  elevations  and  sinking  into  depressions. 
When  the  retina  is  detached,  the  amount  of  light  reflected  from  the  cho- 
roid is  greatly  reduced,  consequently,  the  retinal  vessels  appear  dark  or 
even  black.  They  are  also  somewhat  distended  and  tortuous  from  ob- 
structeci  circulation  due  to  abnormal  bends  and  curves.  The  tendency  of 
spontaneous  detachment  is  to  become  complete,  in  which  event  the  retina 
is  entirely  separated  except  at  the  nerve  and  ora  serrata.  In  total  detach- 
ment if  a  small  vitreous  cavity  remains,  the  retina  will  form  a  funnel-shaped 
tube,  with  bulging  walls,  at  the  bottom  of  which  it  may  be  possible  to  see 
the  optic  disc. 

Frequently  a  rent  is  seen  in  the  detachment  through  which  appears  the 
bright  red  reflex  from  the  choroid.  The  laceration  usually  occurs  above, 
near  the  periphery,  presenting  an  irregular  opening  to  which  may  be  at- 
tached a  flap  of  incurving  retina.  This  rent  is  supposed  to  be  an  important 
factor  in  the  mechanism  of  detachment;  it  certainly  is  present  in  a  large 
proportion  of  all  cases  although  it  may  be  so  situated  as  to  escape  ob- 
servation. Any  portion  of  the  retina  may  become  detached,  but  in  all  cases 
the  subretinal  fluid  tends  to  gravitate  to  the  bottom  of  the  eye,  consequently, 
old  detachments  are  situated  below.     This  is  the  most  favorable  location. 


Fig.  103 — Section  of  Retina  Destroyed  by  Hemorrhage.     The  Blood  has  been  Removed 
and   Pigment-laden    Leucocytes   are    seen   passing   out   into    the   Vitreous. 


Fig.   104. 
Fig.   10-4 — Closure   of  a   Branch   Retinal   Vein.     Hemorrhagic   Retinitis    (to   the   right), 

limited  to  the   Region   Trained  by  the   Occluded  Vessel. 


Fig.   10.".. 

Fig.    105  —  Typical    Detachment    of    the 
Retina. 


Fig.   106. 

Fig.    106  —  IDetachment    of    Retina    and 
Choroid. 


DETACHMENT    OF    THE    RETINA.  163 

as  preser\  ation  of  the  upper  half  of  the  retina  enables  the  patient  to  see 
objects  in  the  lower  field.  Detachment  of  the  macula  is  rare.  When  it 
occurs  the  picture  may  resemble  that  produced  by  closure  of  the  central 
artery,  i.  e.,  a  bright  red  macula  surrounded  by  white  opaque  retina.  The 
red  spot  is  the  choroid  seen  through  the  thin  or  open  fovea.  When  hemor- 
rhage or  exudation  are  present  in  a  detached  retina,  nephritis  or  other 
angiopathic  disease  should  be  suspected.  One  case  of  retinal  pigmentation 
after  detachment  has  been  reported. 

The  symptoms  of  detachment  are  not  always  uniform.  The  surface 
tremor,  so  marked  in  large  flaccid  detachments,  may  scarcely  be  noticeable 
in  those  of  moderate  size  where  the  retina  is  tense.  Occasionally,  detach- 
ments remain  transparent  and  fail  to  exhibit  a  grey  color.  Large  de- 
tachments have  been  seen  in  which  the  retina  retained  its  normal  color  and 
fair  vision  was  preserved.*  Such  cases  raise  a  suspicion  that  both  choroid 
and  retina  were  detached  (Fig.  106).  An  authentic  case  of  chorioretinal 
detachment  the  borders  of  which  were  shelving,  is  recorded  by  Spicer.  The 
retina  was  not  opaque  but  contained  clouded  areas.  The  summit  of  the 
separated  retina  was  elevated  4  mm.  At  this  level  the  choroidal  ves- 
sels Avere  distinctly  seen.     The  visual  field  was  not  contracted. 

Stereo(]RAm  51.  Detachment  of  the  Retina.  Recent  and  of 
high  degree.  Left  fundus  oculi  of  a  man  60  years  of  age.  The  eye  is 
myopic  (-9.  D.)  Two  weeks  ago,  after  violent  vomiting,  the  left  eye  be- 
came nearly  blind.  At  the  present  time,  vision  in  the  upper  field  of  this  eye 
is  reduced  to  perception  of  light.  The  pupil  is  moderately  dilated  and  the 
anterior  chamber  deepened.  On  illuminating  the  fundus  with  the  ophthal- 
moscope, the  upper  half  presents  a  normal  red  color,  but  when  the  patient 
looks  down  the  reflex  becomes  light  grev.  Examination  by  direct  and  in- 
direct methods,  shows  high  detachment  of  the  entire  lower  half  of  the 
retina.  The  detached  membrane  is  thrown  into  wavy  folds  which  undulate 
on  movement  of  the  eye.  Above,  where  the  detachment  is  shallow,  the 
retina  has  a  reddish  cast  which  gradually  merges  into  a  grey  color  below 
where  the  separation  between  choroid  and  retina  reaches  a  height  of  6. 
mm.  (18.  D. ).  The  strong  reflection  of  light  from  the  tops  of  the  retinal 
folds  produces  the  characteristic  "  shot  silk  "  appearance.  The  blood 
vessels  crossing  the  detachment  are  very  dark  and  contain  no  central  light 
streak.  As  they  follow  the  folds  in  the  retina,  their  courses  become  very 
irregular  and  apparently  interrupted.     The  optic  disc  is  half  enclosed  by 


*Nettleship :     Hartridge. 


164  THE    FUNDUS    OCULI. 

a  myopic  crescent.  Transillumination  of  the  region  occupied  by  the  de- 
tachment casts  no  shadow  on  the  pupil. 

Diagnosis.  While  no  difficulty  may  be  experienced  in  recognizing  a 
detachment  of  the  retina,  its  character  is  not  always  easy  to  diagnosticate. 
It  is  of  v'ital  importance  to  differentiate  simple  detachment  from  detach- 
ment secondary  to  an  intraocular  tumor.  In  doubtful  cases,  a  history  of 
injury  or  the  presence  of  myopia,  favors  a  diagnosis  of  simple  detachment. 
As  a  rule,  simple  detachment  occurs  rapidly  while  that  due  to  sarcoma  de- 
velops slowly.  In  transillumination,  simple  detachment  offers  no  obstruc- 
tion to  the  passage  of  light,  in  which  respect  it  differs  from  sarcoma  which 
casts  a  shadow  on  the  pupil.  Eye  tension  may  be  diminished  in  simple 
detachment  but  is  increased  or  normal  in  intraocular  growths.  In  simple 
detachment  vision  may  improve  from  temporary  replacement  or  change  in 
position  of  the  detachment,  while  sarcomatous  detachment  grows  pro- 
gressively worse.  Retinal  detachment  due  to  tumors  may  or  may  not 
present  a  characteristic  appearance.  Sarcoma  of  the  choroid  produces 
a  rounded  elevation  which  projects  abruptly  into  the  vitreous  (Stergms. 
74-75-78).  Usually,  the  retina  lies  in  close  contact  with  the  summit  of  the 
neoplasm  or  is  separated  from  it  by  only  a  thin  layer  of  fluid.  The  sur- 
face of  the  retina  is  smooth,  free  from  folds  and  does  not  tremble  when  the 
eye  moves.  The  color  of  the  sarcoma,  which  may  be  white,  blackish  brown 
or  grey,  often  is  ^'isible  through  the  detachment.  In  other  cases,  the  layer 
of  fluid  between  the  growth  and  retina  is  sufficient  in  depth  to  obscure  the 
true  cause  of  detachment.  Sarcoma  may  produce  extensive  detachment  of 
the  retina  in  regions  remote  from  the  tumor.  Therefore,  in  every  case  of 
detached  retina,  search  should  be  made  for  intraocular  growth.  Detach- 
ment may  be  due  to  sarcoma  and  yet  be  wholly  separated  from  it  (Par- 
sons). This  is  especially  true  of  small  sarcomas  situated  near  the  hor- 
izon, in  which  case  the  detachment  may  be  entirely  posterior  to  the  tumor. 

Metastatic  carcinoma  of  the  choroid  produces  extensive  detachment 
of  the  retina  at  a  much  earlier  stage  of  growth  than  does  sarcoma.  In 
these  cases,  the  usual  situation  of  choroidal  carcinoma  at  the  posterior  pole 
increases  the  difficulty  of  diagnosis. 

Glioma  retinae  is  situated  in  the  retina  itself,  difl^ering  in  this  respect 
from  sarcoma  and  carcinoma  which  are  subretinal  growths.  Owing  to  its 
separation  from  the  sclera,  glioma  does  not  cast  a  shadow  in  transillumina- 
tion. Glioma  endophytum  forms  a  v^ry  evident  neoplasm;  glioma  exo- 
phytum,  on  the  other  hand,  causes  early  and  extensive  detachment  of  the 
retina. 


DiilACHMENT    OF    THE    RETINA.  165 

Conglomerate  tubercle  of  the  choroid  is  prone  to  invade  the  retina, 
and  instead  of  detachment  the  fundus  usually  presents  the  features  of  an 
exudative  process.  Wicherkiewicz,  however,  has  seen  extensive  detachment 
of  the  retina  produced  by  a  tubercular  nodule.  Dor  also  regards  tubercle  as 
a  cause  of  detachment.  If,  in  a  given  case,  tubercle  is  suspected,  the  diag- 
nosis would  be  confirmed  if  an  injection  of  tuberculin  produced  reaction  in 
the   eye. 

Subretmal  cysticercus  forms  a  rounded,  sharply  defined,  translucent  de- 
tachment, beneath  which  it  may  be  possible  to  recognize  the  bluish-grey 
bladder  in  which  the  animal's  head  appears  as  a  light  speck.  The  parasite 
may  be  seen  to  move  within  the  bladder,  but  the  tremulous  motion  charac- 
teristic of  serous  detachment  is  absent. 

Cystic  degeneration  of  the  retina  is  given  as  a  cause  of  detachment. 
Although  large  cvsts  are  found  in  detached  retinas,  it  is  doubtful  if  they 
ever  antedate  detachment.  These  cysts  commence  with  edema  of  the 
nuclear  layer  and  the  bulging  always  is  outward  (Figs.  107-108).  Con- 
sequently,  thev  cannot  be   ophthalmoscopically  recognized   as   cysts. 

The  detached  retina  of  metastatic  retinitis  is  saturated  with  exudates 
which  render  the  membrane  stiff  and  immobile  so  that  it  does  not  tremble 
when  the  eye  is  moved.  The  intraocular  tension  usually  is  diminished  in 
metastatic  retinitis. 

Reattachment.  In  exceptional  cases  reapposition  of  a  detached 
retina  occurs  with  more  or  less  restoration  of  function.  Ophthalmoscopic 
evidence  of  reapposition  usually  exists  in  the  form  of  white  or  pigmented 
streaks  and  spots  situated  behind  the  retinal  vessels.  Such  streaks  con- 
stitute one  form  of  retinitis  striata.  The  striae,  which  usually  corre- 
spond to  the  folds  and  wrinkles  of  the  detached  retina,  may  radiate 
from  the  disc  or  other  points  in  the  fundus.  Frequently,  they  are  ar- 
ranged in  parallel  lines.  In  a  small  proportion  of  cases  the  distribution  is 
irregular.  These  streaks  usually  represent  adhesions  between  the  choroid 
and  retina.  A  previous  detachment,  however,  cannot  be  assumed  from 
the  mere  presence  of  striae.  Another  variety  of  retinal  lines  develops  in  old 
detachments  from  stretching  and  atrophy.  In  commencing  detachment, 
stria*  ciue  to  irregular  light  reflexes  from  wrinkles  in  the  retina  are  common 
(Stergm.  50).  A  section  of  retina  that  has  been  separated  and  reattached 
usually  is  demarcated  from  the  surrounding  fundus  by  grey  or  yellowish 
stripes  which  mav  be  bordered  by  pigment.  Vitreous  membranes  have 
been  observed  in  cases  of  retinal  detachment  associated  with  old  hemor- 
rhages.    The  retinal  epithelium  beneath  a  detached  retina  may  undergo  ir- 


166  THE    FUNDUS    OCULI. 

regular  depigmentation.  In  such  cases,  if  reapposition  occurs,  the  retina  ap- 
pears spotted  as  in  choroiditis.  In  vxry  old  detachments  the  vitreous  may 
contain  crystals  of  cholesterin  and  produce  synchysis.  Cholesterin  is  fre- 
quent also  in  the  subretinal  fluid.  Posterior  cataract  or  phthisis  bulbi  may 
ensue  in  an  eye  in  which  the  retina  has  been  detached  for  years. 

Old  Detachment.  In  course  of  time  a  detached  retina  becomes 
highly  atrophic  and  regains  its  transparency  and,  if  at  the  same  time  the 
subretinal  fluid  is  clear,  the  detached  area  may  again  exhibit  a  red  reflex. 
The  vessels  also  become  lighter  in  color,  although  the  veins  always  remain 
darker  than  normal. 

Stereogram  52.  Detachment  of  the  Retina.  Old,  shallow  and 
translucent.  Left  eyeground  of  girl,  19  years  of  age.  Stenographer.  Re- 
fraction normal.  Four  years  ago  she  was  struck  in  the  left  eye  by  a  ball, 
which  caused  her  to  faint.  Four  months  later,  a  positive  central  scotoma, 
like  a  black  disc,  appeared  before  the  left  eye.  At  that  time  she  experienced 
flashes  of  light,  which  disappeared,  but  recently  have  recurred.  The 
scotoma  gradually  passed  away  and  now,  central  vision  is  20/40.  Per- 
ception of  light  is  abolished  in  the  upper  part  of  the  visual  field  (Fig.  109). 
In  the  left  eye  the  anterior  chamber  is  somewhat  deeper  than  in  the  right. 
With  ophthalmoscopic  illumination  the  fundus  gives  a  uniform  red  reflex. 
With  direct  examination,  the  lower  half  of  the  retina  is  seen  to  be  detached. 
The  separation  commences  on  a  horizontal  line  passing  just  below  the 
disc  and  increases  gradually  until  at  the  equator  it  reaches  an  elevation 
of  3  mm.  (9.  D.).  The  surface  of  the  detachment  is  slightly  undulating. 
The  color  is  nearlvthat  of  the  normal  fundus,  although  faint  light  reflexes 
come  from  the  uneven  surface.  The  blood  vessels  are  somewhat  dilated 
and  follow  the  surface  inequalities  of  the  retina.  The  arteries  are  but  little 
darker  than  those  in  the  upper  part  of  the  fundus,  the  veins  however  are 
nearlv  black.  Just  above  the  upper  margin  of  the  detachment  are  a  few 
horizontal,  yellowish  streaks,  which  are  interpreted  as  representing  ad- 
hesions between  choroid  and  retina  in  a   region   formerly  detached. 

The  historv  indicates  that  detachment  formerly  extended  above  the 
macula  and  that  the  fluid  has  gravitated  below  into  its  present  position. 
That  the  detached  retina  is  atrophic  is  shown  by  its  absolute  blindness  and 
by  its  transparency,  which  permits  the  passage  of  light  reflected  from  the 
choroid.  The  delayed  symptoms  may  be  explained  by  an  unrecognized 
peripheral   detachment  which   suddenly  changed   its  position. 

The  prognosis  in  this  case,  as  regards  extension  of  the  process,  is 
good      The  detachment  was  due  to  traumatism  and  the  cause  is  no  longer 


Fig.  107.  Fig.  108. 

Figs.   107,   108 — Cystic  Formation  in   Retinal   Detachment. 


w>  so  90  y>  io 


no" 


Fig.  109. 


DETACHMENT    OF    THE    RETINA.  167 

active,  differing  in  this  from  detachment  caused  by  a  progressive  disease, 
as  occurs  in  myopia. 

Diagnosis.  Old,  low,  translucent  detachments  may  be  overlooked, 
owing  to  their  red  color,  but  once  discovered  they  can  hardly  be  mistaken 
for  another  condition.  The  course  and  dark  color  of  the  blood  vessels  are 
diagnostic.  I'he  form  and  color  of  the  detachment  shown  in  the  stereogram 
do  not  suggest  the  presence  of  a  tumor. 

SvMPiOMS.  Retinal  detachment  often  is  preceded  by  subjective  symp- 
toms indicating  disturbance  in  the  rod  and  cone  layer.  The  most  common 
are  photopsiae  manifested  by  a  sensation  of  fiery  sparks,  darts,  balls,  etc., 
passing  before  the  eye.  Metamorphopsias  often  are  present  and  objects  ap- 
pear crooked  or  altered  in  size.  Chromotopsias  also  occur,  red,  blue,  brown 
and  violet  visions  being  the  most  common.  A  frequent  complaint  is  of 
clouds  or  dust  before  the  eye  or  of  momentary  loss  of  vision. 

When  these  symptoms  appear  in  myopia  they  should  be  regarded  as 
signals  of  impending  detachment,  although  frequently  they  disappear  with- 
out such  termination.  If,  however,  detachment  ensues  these  phenomena 
may  persist  and  prove  very  annoying.  Detachment  usuallv  occurs  sud- 
denly, the  event  being  signalized  by  the  sensation  of  a  cloud  or  curtain 
before  the  eye.  Functional  disturbance  is  most  noticeable  and  annoying  in 
rapidly  developed  cases  in  which  light  perception  is  often  retained  for 
months  or  even  years.  The  undulating  motions  of  the  retina  cause  images 
which  are  formed  upon  the  affected  region  to  sway  and  wave.  Reduced 
central  vision  usually  exists  even  though  the  macula  is  not  visibly  involved. 
In  some  cases,  during  the  forenoon,  vision  may  be  good  enough  to  locate 
objects  but  later  in  the  day  will  sink  to  mere  perception  of  light.  The 
matudinal  improvement  is  due  to  reposition  of  the  retina  which  occurs  at 
night  while  the  patient  is  recumbent.  Weeks  states  that  in  these  cases  pho- 
topsi-T  always  are  experienced  when  redetachment  occurs.  Besides  the 
chromopsia  described  above,  the  color  sense  may  be  impaired  as  regards 
blue  and  green.  Leber  thinks  this  is  due  to  the  yellow  color  of  the  sub- 
retinal  fluid.  As  tested  by  ordinary  methods,  high  detachments  are  repre- 
senteci  in  the  visual  fields  as  positive  scotomata.  In  case  of  double  de- 
tachment below,  the  fields  will  show  a  bilateral  upward  hemianopsia  (Fig. 
12).  In  very  flat  detachment,  in  which  elevation  cannot  be  demonstrated  by 
the  ophthalmoscope,  torpor  retinae  usually  exists,  and  a  field  which  appears 
normal  in  bright  light  will  exhibit  a  scotoma  when  illumination  is  reduced. 
Such  patients  cannot  see  well  on  cloudy  days.  Detachment  of  the  retina 
occurs  most  frequentlv  between  the  45th  and  60th  years.     It  is  rare  before 


168  THE    FUNDUS    OCULI. 

the  age  of  thirty.  As  a  rule,  one  eye  only  is  affected  although  binocular 
cases  are  not  unusual.  Hortsman  reported  106  cases  of  spontaneous  detach- 
ment of  which  9  occurred  in  both  eyes. 

Prognosis.  The  prognosis  of  retinal  detachment  in  myopia  is  very 
bad,  but  where  it  is  due  to  Bright's  disease  or  to  traumatism,  reposition 
of  the  retina  and  restoration  of  vision  may  occur.  The  rods  and  cones 
are  very  resistant  to  pathological  conciitions  and,  if  the  retina  can  be  re- 
placed within  a  few  weeks  or  months  after  detachment,  they  may  resume 
their  function.  With  the  development  of  atrophy,  however,  light  percep- 
tion is  lost  and  nothing  would  be  gained  were  the  retina  once  more  in 
contact  with  the  choroid.  Notwithstanding  published  reports,  the  per- 
ma^nent  cure  of  a  detached  retina  is  a  rare  event.  Although  temporary 
replacement  may  occur,  redetachment  ensues  in  nearly  all  cases.  Uthoft 
collected  all  cases  ol  spontaneous  retinal  detachment  in  medical  literature 
reported  as  cured.  Of  these,  24  per  cent  had  operativ^e  treatment,  45 
per  cent  non-operative  treatment  and  31  per  cent  no  treatment  whatever. 
These  statistics  render  it  difficult  to  estimate  the  influence  of  treatment  in 
reducing  detachment  of  the  retina,  inasmuch  as  many  of  those  treated 
might  have  recovered  spontaneously.  The  best  results  from  treatment  are 
obtained  in  albuminuria  with  general  anasarca.  Uthoft  reported  a  case  of 
nephritis  with  general  retinal  detachment  in  both  eyes,  in  which  re- 
attachment followed  drainage  of  the  dropsical  fluid  through  the  legs  by 
Cruschmann's  canulas.  A  detachment  which  does  not  increase  in  size 
must  be  regarded  as  having  terminated  favorably. 

Etiology.  The  cause  of  serous  detachment  of  the  retina  is  unknown 
despite  the  frequent  opportunities  presented  for  clinical  and  anatomical 
study  of  the  affection.  Several  opposing  theories  exist,  any  of  which  may 
be  true  for  certain  cases  but  not  one  of  them  satisfactorily  explains  all  the 
» features  presented  by  the  most  common  type  of  spontaneous  detachment. 
The  majority  of  cases  occur  in  myopia.  Uthoff  found  In  500  cases  of 
spontaneous  detachment  that  60  per  cent  were  myopic.  He  found  also 
that  detachment  occurs  in  4.5  per  cent  of  all  cases  of  myopia  in  adults. 
Statistics  showing  a  lower  percentage  include  children,  among  whom  de- 
tachment is  extremely  rare.  Detachment  of  the  retina,  however,  is  not  pe- 
culiar to  myopia  but  occurs  also  in  both  emmetropia  and  hypermetropia. 
It  is  not  a  rare  affection.  Schobl,  among  120,000  cases  of  eye  disease, 
found  280  cases  of  retinal  detachment. 

Following  are  the  principal  theories  which  have  been  advanced  to 
explain  the  development  of  simple  retinal  detachment.     They  all  possess 


DETACHMENT    OF    THE    RETINA.  169 

a  logical  basis  and  any  of  them  may  be  partly  or  wholly  applicable  to 
certain  cases. 

ExiDATiox  OR  Secretion  Theory.  This  theory  was  conceived  by 
Arlt  and  is  supported  by  Schmidt-Rimpler.  The  choroid  is  supposed  to 
furnish  a  fluid  which  accumulates  behind  the  retina.  The  retina  is  pushed 
forward  against  the  vitreous,  causing  the  latter  to  be  liquified  and  absorbed. 
An  associated  disease  of  the  ciliary  body  is  supposed  to  be  present  which 
causes  further  shrinking  of  the  vitreous  and  increases  the  retinal  detach- 
ment. Probablv  this  explanation  is  true  for  certain  cases  of  flat  detach- 
ment; for  example,  I  have  seen  a  young  man  in  whom  a  low  degree  of 
detachment  slowly  d2\eloped  in  both  eyes.  1  he  surface  of  the  separated 
retina  was  smooth  and  exhibited  no  tremor.  Such  a  condition  was  explained 
best  by  the  theory  of  choroidal  exudation.  There  is  little  doubt  that  the 
retinal  detachment  which  occurs  in  albuminuria  is  a  manifestation  of  edema 
in  which  the  transudate  comes  from  the  choroidal  vessels.  In  this  disease 
detachment  may  come  and  go  with  the  general  anasarca  as  in  Lthoff's 
case  described  above,  or  in  Raehlmann's  in  which  chemosis  of  the  bulbar 
conjunctiva  and  retinal  detachment  appeared  and  disappeared  together. 
Raehlmann's  case  is  of  further  interest  as  it  occurred  in  a  case  of  nephritis 
in  which  there  was  no  retinitis.  Galezowski  regards  .rheumatism  as  a  fre- 
quent cause  of  detachment  and  in  such  cases  he  thinks  there  is  an  effusion 
from  the  choroid  analogous  to  that  which  is  poured  into  the  joints.  The 
exudation  theory  is  particularly  applicable  to  separation  of  the  retina 
which  sometimes  develops  during  the  course  of  nasal  sinusitis  and  orbital 
abscess  and  which  disappears  w^hen  the  suppurative  process  is  cured.  The 
detachment  which  accompanies  subretinal  tumors  appears  to  be  caused  by 
a  fluid  which  the  choroid  exudes  in  response  to  the  irritating  action  of  the 
neoplasm.  It  has  been  suggested  that  the  detachment  which  often  results 
from  application  of  blunt  force  to  the  eye  may  be  caused  by  exudation  from 
the  choroid.  In  the  exudative  stage  of  choroiditis  an  effusion  often  appears 
between  the  retina  and  choroid.  This  condition  frequently  is  seen  in  patho- 
logical specimens  but,  clinically,  its  recognition  is  uncertain.  Stereogram 
18  shows  an  elevated  area  of  the  fundus  in  which  it  is  assumed  that  the 
retina  is  lifted  by  choroidal  exudation.  In  panophthalmitis  the  retina  is 
detached  by  purulent  exudates  from  both  retina  and  choroid  (Fig.  32). 
A  subretinal  exudate  from  the  choroid  is  an  essential  factor  in  the  diffusion 
theory  of  Raehlmann,  vide  infra.  Cyclitic  disease  which  is  mentioned  in 
connection  with  the  exudation  theory  may  yet  be  shown  to  exert  a  greater 
influence  in  causing  detachment  than  is  generally  supposed. 


)70  THE    FUNDUS    OCULI. 

Although  the  exudative  theory  holds  true  for  the  class  of  cases  de- 
scribed above,  strong  objections  exist  to  the  view  that  spontaneous, 
serous  detachment  is  caused  by  exudation  from  the  choroid.  In  most  cases 
retinal  detachment  occurs  rapidly  or  even  suddenly,  and  it  is  inconceivable 
that  the  contents  of  the  globe  could  suddenly  be  so  greatly  increased  without 
raising  intraocular  pressure.  Furthermore,  there  is  no  evidence  that  the 
choroid  is  capable  of  suddenly  producing  the  large  quantity  of  fluid  con- 
tained in  the  subretinal  space  of  a  large  detachment. 

Stretching  or  Mechanical  Theory.  The  frequent  occurrence 
of  retinal  detachment  in  high  axial  myopia  led  von  Graefe  to  the  conclusion 
that  separation  of  the  retina  in  such  cases  was  caused  by  anterio-posterior 
elongation  of  the  eyeball.  This  view  was  supported  also  by  Donders.  The 
mechanism  of  this  theory  is  as  follows.  In  myopia  the  posterior  segment 
of  the  sclera  is  displaced  backward,  dragging  with  it  the  adherent  choroid. 
The  retina,  however,  is  not  adherent  to  the  choroid,  consequently,  slight 
causes  mav  interrupt  its  backward  excursion  and  the  two  membranes  may 
separate.  The  tendency  to  separation  is  increased  also  by  fluidity  of  the 
vitreous  and  hyperemia  of  the  choroid. 

This  theory  is  applicable  to  myopic  cases  only,  but  in  these  it  should 
be  given  great  consideration.  Axial  myopia  predisposes  to  detachment  of 
the  retina,  the  higher  the  myopia  the  greater  the  predisposition.  The  full 
explanation  for  this  may  not  be  forthcoming  but,  whatever  other  causes 
exist,  it  must  be  conceded  that  mere  elongation  of  the  globe  creates  con- 
ditions highly  favorable  to  detachment.  Thus,  the  posterior  portion  of  the 
retina  is  connected  only  at  the  optic  nerve,  therefore,  if  this  membrane  is 
to  follow  the  choroid  and  sclera  it  must  be  pushed  backward  by  an  increase 
in  the  volume  of  the  vitreous.  The  requisite  increase  of  fluid  is  readily  sup- 
plied by  the  normal  cells  of  the  ciliary  processes,  but  if  these  cells  are 
diseased  the  volume  of  the  vitreous  will  not  be  increased  and  the  retina  will 
not  be  held  against  the  choroid.  Myopia  tends  to  produce  detachment  of 
the  retina  by  its  action  on  the  vitreous.  The  inert  vitreous  framework 
does  not  increase  in  size;  on  the  contrary,  stretching  causes  the  fibers  to 
rupture  and  disintegrate,  leavnng  the  vitreous  in  a  fluid  state.  Another 
result  of  stretching  is  thinning  and  tearing  of  the  retina  which  renders  it 
easy  for  a  fluid  vitreous  to  filter  through  and  produce  detachment. 

Hemorrhagic  Theory.  Because  of  its  sudden  development,  von 
Graefe  once  thought  that  retinal  detachment  must  be  due  to  hemorrhage, 
but  he  soon  abandoned  this  view.  Of  course,  hemorrhage  from  the  choroid 
no  longer  is  advanced  as  the  cause  of  simple  detachment,   but  it   holds 


Fibers  of  the  Vitreous,  after   Abstraction   of   its    Watery   Content   by   the   Process   of 

Hardening. 
f 


Fig.   111. 

Fig.   Ill — Large,  clear  space  left  in  front  of  the  Retina  by  Contraction  of  Pathologic. 
Albuminous  Vitreous  during  Process  of   Hardening. 


DEIACKMENT    OF     1111".    RKTINA.  171 

good  for  certain  cases  of  a  hemorrhagic  type  which  occur  in  vascular 
disease    and   after   Injuries   or   operations    (Figs.    115-116). 

Detachment  of  the  Vitrfous.  Separation  of  the  vitreous  from 
the  retina  was  first  described  as  a  pathologic  process  by  H.  Mijller. 
Later,  Iwanoff  describeci  localized  posterior  detachment  of  the  vitreous, 
in  which  a  cavity  containing  (luid  is  supposed  to  form  between  the  vitreous 
and  retina.  In  myopia  this  cavity  is  further  increased  by  elongation  of 
the  eveball.  The  fluid  content  of  the  cavity  is  said  to  increase  until  the 
pressure  becomes  so  great  that  the  fluid  filters  through  the  retina  and 
lifts  it  from  the  choroici.  DeWecker  accepted  the  post  vitreous  spaces, 
but  thought  the  presence  of  fluid  forced  a  hole  through  the  retina. 

This  theorv  of  the  mechanism  is  faulty.'  A  collection  of  fluid  as 
conceived  bv  Iwanoff  would  exert  equal  pressure  in  all  directions  through 
the  soft  vitreous,  moreover,  increased  pressure  tends  to  hold  the  retina  in 
place,  not  to  detach  it.  Despite  the  apparent  improbability  of  this  theory, 
the  microscopic  picture  of  vitreous  detachment,  as  described  by  Iwanoff, 
has  repeatedly  been  confirmed  by  other  investigators  and  thus  has  acquired 
pathologic  standing.  Nevertheless,  it  is  extremely  doubtful  that  such  a  con- 
dition actually  exists  hitra  vitani.  Greeft,  Elschnig  and  others  who  have 
carefully  investigated  this  subject,  are  emphatic  in  declaring  that  the 
so-called  vitreous  detachment  is  a!i  artefact  produced  by  shrinkage  when 
the  eye  is  hardened  for  microscopic  examination.  The  justice  of  this 
criticism  is  apparent  to  those  who  frequently  dissect  human  eyes.  The 
normal  vitreous  is  about  99  per  cent  water,  all  of  which  is  abstracted 
when  the  eye  is  hardened,  lea\ing  nothing  in  the  vitreous  cavity  except 
fibers  (Fig.  110).  Under  pathologic  conditions,  however,  the  vitreous 
contains  more  or  less  albumen  which  forms  a  visible  coagulum  in  the  pre- 
pared eye.  This  albuminous  coagulum  mav  contract  from  behind  forward, 
leaving  large  clear  spaces  posteriorly  (Fig.  Ill  ).  Examination  of  frozen 
eyes  has  cast  still  further  doubt  upon  the  existence  of  vitreous  detachment 
in  the  living  eye.  Examination  of  my  own  specimens  would  indicate  that 
the  so-called  detachment  of  the  vitreous  is  not  present  In  myopic  eyes  or 
eyes  with  recent  detachment  of  the  retina.  If,  however,  in  these  or  other 
eyes,  the  vitreous  Is  moderately  albuminous,  artefacts  resembling  the  vit- 
reous spaces  of  Iwanoff  will  be  found. 


172  THE    FUNDUS    OCULI. 

Retraction  Theory  (Fibrillary  Degeneration  of  the  Vitreous). 
This  theory,  which  was  presented  by  Leber  and  Nordenson,  attributes 
detachment  of  the  retina  primarily  to  a  fibrillary  degeneration  of  the 
vitreous,  consisting  of  an  increase  in  the  thickness  of  the  fibers  which 
form  its  framework.  These  thickened  fibers  are  supposed  to  undergo  con- 
traction and  produce  detachment  of  the  vitreous,  leaving  a  space  between 
it  and  the  retina  filled  with  Huid.  The  final  mechanism  of  detachment  is 
given  as  follows.  Progressive  contraction  of  the  vitreous  fibers  attached  to 
the  anterior  portion  of  the  retina  tear  a  hole  in  the  latter  through  which 
the  post-hvaloid  fluid  passes  and  detachment  is  accomplished.  By  this 
process  the  retina  may  be  separated  slowly  or  rapidly  without  increasing 
intraocular  pressure,  because  the  contents  of  the  eye  are  not  increased,  the 
fluid  simply  changing  position.  During  all  these  changes  the  vitreous 
fibers  remain  invisible  and  the  vitreous  does  not  lose  its  transparency. 

Leber  and  Nordenson's  theory,  which  is  widely  accepted,  is  extremely 
plausible.  It  is  founded  upon  the  most  conscientious  laboratory  work  and 
every  microscopic  detail  described  can  be  confirmed  in  the  human  eye  and 
in  the  eyes  of  animals  in  which  the  retina  has  been  artificially  detached. 
LInfortunately,  however,  it  appears  that  all  the  pathologic  findings  in- 
cluding fibrillary  vitreous,  vitreous  fibers  and  post-hyaloid  spaces,  can  be 
found  in  any  eye  with  a  moderately  albuminous  vitreous,  which  has  been 
subjected  to  the  laboratory  methods  of  hardening.  Especially,  is  this 
true  in  mildly  inflammatory  conditions  in  which  the  fibrin  coagulates  into 
a   fine   network  and  deposits   appear  upon   the   vitreous  fibers. 

The  validity  of  the  retraction  theory  depends  upon  proving  that 
the  vitreous  fibers  possess  the  power  of  proliferation.  The  vitreous  con- 
tains few,  if  any,  fixed  cells  and,  as  asserted  by  Circione,  Greeff  and  others, 
it  is  improbable  that  the  vitreous  fibers  are  capable  of  reproduction,  re- 
generation or  enlargement  and,  furthermore,  the  only  change  they  ever 
undergo  is  to  disappear.  According  to  Retzius  the  vitreous  framework 
tends  to  break  down  in  adult  life  and  give  place  to  spaces  containing  a  thin 
fluid,  and  in  the  aged  the  vitreous  may  be  entirely  liquified.  The  condition 
described  in  pathological  reports  as  fibrillary  degeneration  of  the  vitreous 
is  a  normal  condition  produced  in  the  following  manner.  The  vitreous  is 
a  jelly-like  body  about  99  per  cent  of  which  is  water.  The  fluid  is  en- 
closed in  an  extremely  delicate  membrane  and  supported  by  a  tangled 
mesh  of  long,  delicate  fibers  attached  mostly  in  front.  After  an  eye  has 
been  hardened  and  the  water  extracted,  these  fibers  are  seen  streaming 
out  into  the  vitreous  cavity  in  long  tufts  like  a  horse's  tail  (Retzius)    (Fig. 


DETACHMENT    OF    THE    RETINA.  173 

110).  They  are  In  no  sense  pathologic,  but  if  inflammatory  conditions 
exist,  exudates  and  albuminous  matter  may  be  deposited  on  them.  Raehl- 
mann  denies  that  the  findings  of  Leber  and  Nordenson  are  convincing, 
inasmuch  as  the  eyes  were  examined  long  after  detachment  occurred  and 
the  vitreous  changes  probably  developed  after  separation  of  the  retina. 
The  retina  is  not  readily  drawn  from  its  bed  and  it  is  incomprehensible  that 
this  can  be  accomplished  by  bands  in  the  vitreous  which  are  ophthalmo- 
scopically  invisible.*  Highly  developed  fibers  and  membranes  in  the  vit- 
reous seldom  lead  to  retinal  detachment  so  long  as  the  ciliary  body  supplies 
sufficient  fluid  to  support  the  vitreous  and  maintain  normal  intraocular 
pressure.  Fig.  112  represents  one  of  several  specimens  which  I  have 
examined  illustrating  this  fact.  Furthermore,  retinal  detachment  may 
occur  in  myopia  with  no  demonstrable  change  in  the  vitreous.  In  the 
case  of  ciliary  telangiectasis  (p.  288-289)  the  eye  was  enucleated  two  weeks 
after  spontaneous  detachment.  On  microscopic  examination  not  the  slight- 
est evidence  of  so-called  fibrillary  degeneration  of  the  vitreous  was  present; 
on  the  contrarv,  the  vitreous  fibers  were  unusually  scanty  (Fig.  113). 
Even  shrinking  of  the  vitreous  from  contraction  of  inflammatory  exudates 
does  not  invariably  detach  the  retina   (Fig.   112). 

The  retraction  theory  requires  that  the  retina  be  torn,  but  Nordenson 
found  rents  in  but  38  per  cent  of  his  cases.  In  the  discussion  following 
Leber's  presentation  of  the  retraction  theory,  Samelsohn  stated  that  he 
had  seen  a  rupture  appear  on  the  summit  of  a  detachment  coincident  with 
improvement.  The  detachment  which  usually  follows  violent  Iridocyclitis 
and  shrinking  of  the  vitreous,  Is  a  process  quite  distinct  from  the  fibrillary 
degeneration  and  detachment  of  Leber  and  Nordenson. 

Diffusion  Theory.  Raehlmann  affirms  that  retinal  detachment  is 
the  result  of  simple  osmosis.  To  construct  this  theory  he  assumes  the 
existence  of  the  following  conditions:  (1)  A  highly  albuminous  exudate 
from  the  choroid,  situated  behind  the  retina;  (2)  A  faintly  albuminous, 
fluid  vitreous  In  front  of  the  retina.  Here  are  two  fluids  of  different  density 
separated  by  the  thin  retina  which  acts  the  part  of  a  dialysing  membrane. 
When  these  two  fluids  differ  greatly  in  density,  diffusion  will  be  rapid; 
when  the  difference  Is  slight,  diffusion  will  be  slow.  In  accordance  with 
physical  law,  the  n^ost  rapid  flow  is  toward  the  subretlnal  space,  causing 
there  a  great  increase  of  pressure.     At  the  same  time,  the  vitreous  cavity. 


*  Greeflf  truly  says,  "  the  cicatricial  bands  which  Deutschmann  severs  in  his  opera- 
tion for  the  cure  of  detachment,  have  never  been  seen  either  opthalmoscopically  or  micro- 
scopically,  nor   have   they  been    felt   with   the  knife." 


174  THE    FUNDUS    OCULI. 

having  parted  with  more  fluid  than  it  has  received,  will  have  its  pressure 
reduced.  The  high  pressure  behind  the  retina  is  supposed  to  burst  the 
membrane,  causing  the  rents  and  fissures  seen  in  detachment,  while  the 
low  pressure  in  the  vitreous  cavity  explains  the  retraction  of  the  lens,  deep 
anterior  chamber  and  diminished  tension  of  old  retinal  detachment.  The 
frequent  absence  of  lacerations  in  retinal  detachment  is  urged  by  RaehJ- 
mann  as  an  argument  in  favor  of  the  diffusion  theory  and  against  that  of 
retraction.* 

The  diffusion  theory  is  very  attractive  and  meets  the  requirements 
better  than  any  other  which  has  been  advanced.  Nevertheless,  it  is  not  free 
from  criticism.  It  is  difficult  to  accept  it  for  those  cases  in  which  detachment 
occurs  almost  instantaneously.  The  theory  also  shows  weakness  in  elabora- 
tion of  details.  Thus,  if  diminished  eye  tension  is  due  to  difference  in 
pressure  between  the  vitreous  cavity  and  subretinal  space,  why  does  low 
tension  continue  when  the  retina  is  torn  and  the  two  cavities  communicate? 

That  subretinal  accumulations  of  fluid  do  occur  in  myopia  is  shown 
in  Fig.  160.  In  this  eye  there  was  not  the  slightest  tendency  for  the 
retina  to  become  detached.  Neither  did  the  vitreous  contain  the  usual 
number  of  fibrils. 

Hyperemic  Theory.  One  other  theory  appears  worthy  of  mention, 
namely,  that  of  Riva.  This  author  considers  that  congestion  of  the  cho- 
roid alone  is  sufficient  to  detach  the  retina.  Although  choroidal  hyperemia 
appears  inadequate  to  explain  serous  detachment,  it  is  a  matter  of  common 
observation  that  separation  of  the  retina  often  follows  coughing,  vomiting, 
sneezing,  lifting  or  anything  which  produces  sudden  congestion  of  the  head. 
In  these  cases  it  is  fair  to  assume  that,  when  other  conditions  favor  de- 
tachment, the  determining  factor  is  congestion  of  the  choroid.  Congestion 
may  be  an  important  element  also  in  the  detachment  that  follows  appli- 
cation of  blunt  force  to  the  eye.  A  variation  of  the  hyperemic  theory  is 
advanced  by  Dor,  who  thinks  that  in  certain  diseases  the  blood  pressure  is 
diminished  in  the  ciliary  body  and  increased  in  the  choroid,  conditions  favor- 
able to  retinal  detachment. 

Iridocyclitis.  In  this  disease  it  is  demonstrable  that  the  retina  is 
dragged  inward  by  contraction  of  inflammatory  bands.  Inflammatory  ex- 
udates are  deposited  in  the  anterior  part  of  the  vitreous,  become  vascu- 
larized and  converted  into  strands  of  fibrous  tissue  attached  to  the  ciliary 
body  and  retina  ( Fig.  114).    Contraction  of  this  tissue  leads  to  detachment 


*Von  Graefe  suggested  pressure  of  a  subretinal   exudate  as  a  cause  for  detachment 


Fig.  ll:i — S'lirinka.^^e  of  \'itrcou.s  (V) 
from  Inflammatory  Exudates  vvit.io.;t 
Detachment  of  the  Retina.  The  A]5- 
parent,  Shght  Detacliment  is  an  Artefact. 


Fig.  112 


Fig.  113 — Spontaneous  Detachment  of 
the  Retina.  Tlie  Vitreous  Fibers  are 
Scanty. 


Fig.   113. 


Detachment  of  the  Retina  due  to  the  Con- 
traction of  Bands  of  Fibrous  Tissue  Formed 
by  Iridocylitis. 


FiR.  114. 


Fig.  115 — Detachment  of  the 
Retina  Following  Hemorrhage  into 
the  Choroid,  Probably  Due  to 
Thrombosis  of  the  Vena  Vorticosae. 


Fig.  116 — Choroidal  Hemorrhage 
Causing    Retinal    Detachment. 

S.  C,  Sclerotic  choroid;  B,  blood; 
Scl.   Sclera. 


Fig.  ni;. 

Fig.  117 — Congenital  Detachment 
of  the   Retina. 

The  Retina  is  Markedly  Thick- 
ened by  Exudates  and  is  Adherent 
to  the  Ciliary  Body.  The  Aqueous 
is  Transparent.  The  Retinal  De- 
tachment was  Probably  Caused  by 
Metastatic  Endophthalmitis. 

(Specimen  of  Dr.  A.  E.  Davis.) 


dp:tachmknt  of  thf.  rktina.  175 

of  the  retina.  Progressiv^e  softening  and  shrinking  of  the  globe  ensue, 
constituting  the  condition  known  as  phthisis  bulbi.  Probably,  the  fluid 
portion  of  the  vitreous  is  secreted  entirely  by  those  cells  of  the  pars  cil- 
Uar'is  retiiue  which  extend  from  the  ora  serrata  to  the  cihary  processes.  In 
cyclitis,  these  cells  sutler  and  the  vitreous  is  altered  in  composition  and 
diminished  in  (juantity.  In  some  cases  of  phthisis  bulbi,  the  presence  of 
adventitious  bands  attached  to  the  retina  is  not  demonstrable.  In  such 
cases  it  is  probable  that  detachment  of  the  retina  is  due  to  the  amount  of 
vitreous  being  insuiiicient  to  hold  the  retina  in  position. 

Hkmokkh  \c;ic  Di  iachmkxt  of  thf  Rftina.  This  is  a  clinical 
classification  including  only  cases  of  subretinal  hemorrhage  in  which  the 
retina  is  not  ruptured  or  the  vitreous  filled  with  blood.  The  bleeding  may 
come  from  either  the  retina  or  choroid.  Ophthalmoscopically,  the  detach- 
ment has  a  dark  brown  or  black  tint.  It  occurs  usually  in  the  subjects  of 
arteriosclerosis,  although  Schobl  states  that  such  hemorrhages  are  seen  also 
in  anemic  youths  and  chlorotic  girls.  I  have  microscopically  examined  one 
such  case  which  occurred  in  a  man,  40  years  of  age,  affected  with  general 
arteriosclerosis.  The  retina  was  detached  by  bleeding  into  the  choroid. 
The  source  of  hemorrhage  appeared  to  be  one  of  the  vena  vorticose  which 
was  closed  by  a  fibrous  plug,  probablv  an  organized  thrombus  (Figs. 
115-116). 

Traumatism.  Detachment  frequentlv  follows  blows  to  the  eye,  brow 
or  temple.  Galezowski  says  that  weeks  or  months  may  intervene  between 
the  blow  and  resulting  detachment.  This  fact  has  an  important  medico- 
legal bearing.  These  cases  usually  are  myopic,  but  the  amount  of  myopia 
is  unimportant,  a  blow  producing  detachment  with  equal  readiness  in 
either  low  or  high  degrees. 

Detachment  of  the  retina  may  be  congenital.  J.  S.  Fernandez  has 
reported  an  instance  in  which  two  out  of  fiv^e  children  in  one  family  were 
born  with  total  detachments  in  both  eyes.  Svphilis  was  not  established  as 
a  cause.  From  the  descriptions,  most  of  the  published  cases  appear  to  be 
examples  of  metastatic  retinitis.  I  have  microscopically  examined  similar 
congenital  cases  in  which  the  retina  was  totally  detached  and  matted 
together  bv  exudates,  while  the  aqueous  and  lens  were  perfectly  transparent 
(Fig.  117). 

Certain  svstemic  diseases  have  been  regarded  as  bearing  a  causative 
relation  to  detachment  of  the  retina.  Galezowski,  whose  experience  with 
this  affection  is  enormous,  regards  rheumatism  as  one  of  the  chief  causes. 
Among   1158  cases  of  spontaneous  detachment  he  found  an  arthritic  di- 


176  THE    FUNDUS    OCULI. 

athesis  in  732.  Good  reasons  appear  for  attributing  some  cases  to  syphilis. 
Nephritis,  undoubtedly  is  causative.  Detachment  has  been  attributed  also 
to  diabetes,  malaria,  erysipelas  and  infectious  diseases,  but  relationship 
to  these  affections  remains  to  be  established.  A  patient  of  Bellecontre's 
with  Grave's  disease,  had  detachment  after  division  of  the  cervicle  sympa- 
thetic. Schirmer  reports  the  case  of  a  girl  with  slight  myopia  who  suffered 
detachment  of  the   right   retina   during  chloroform   anesthesia. 

The  various  theories  which  have  been  discussed  in  the  preceding 
pages  agree  in  this:  that  both  myopia  and  fluidity  of  the  vitreous  strongly 
predispose  to  detachment  of  the  retina.  Myopia  produces  fluidity  of  the 
vitreous,  but  fluidity  may  dev^elop  also  in  non-myopic  eyes.  The  mech- 
anism of  serous  detachment  appears  to  consist  of  a  fluid  passing  from  the 
vitreous  cavity  into  the  subretinal  space.  It  is  highly  probable  that  this 
transposition  of  fluid  occurs  in  various  ways.  It  is  easy  to  understand  how  it 
may  trickle  through  an  atrophied  retina  or  pour  through  a  rent.  Under 
proper  conditions  the  diftusion  theory  of  Raehlmann,  undoubtedly,  would 
be  operative.  If  tangible  fibers  exist  in  the  retina,  as  after  inflammation  or 
penetrating  wounds,  their  contraction  would  produce  detachment.  A  more 
careful  study  should  be  made  in  detachment  of  those  cells  which  furnish 
the  vitreous  fluid.  Another  class  of  detachments,  not  always  clinically 
distinguishable  from  serous  detachment,  appear  to  be  caused  by  exudation 
from  the  choroid. 

The  pathologic  anatomy  of  retinal  detachment  has  been  described 
mostly  in  connection  with  its  etiology.  A  few  disconnected  details,  how- 
ever, remain  which  require  mention.  The  majority  of  microscopic  examin- 
ations have  been  made  in  old  cases  in  which  degenerative  changes  in  the 
retina  and  vitreous  may  have  been  secondary  to  separation  (Figs.  118-119). 

T  have  examined  one  case  enucleated  two  weeks  after  spontaneous 
detachment  (page  288-289).  The  vitreous  and  subretinal  fluid  contained 
very  little  albumin.  Vitreous  fibers  were  scanty  (Fig.  113).  Not  the 
slightest  trace  of  "  fibrillary  degeneration  "  of  the  vitreous  was  discovered. 
The  detachment  was  extensive  and  included  the  macula.  The  detached  por- 
tion of  retina  was  edematous.  In  the  macular  region  and  at  the  fovea,  the 
rod  and  cone  layer  were  well  preserved  (Fig.  120).  There  was  neither 
exudation  from  the  choroid  nor  evidence  of  inflammatory  action  in  any 
part  of  the  eve. 

Saltzman  found  defects  in  the  lamina  elastica  of  the  choroid  in 
myopia.  Like  all  so-called  elastic  membranes,  this  layer  does  not  stretch 
well,   and  when  the  eye  elongates  it  becomes  fissured.      Fluid   from   the 


Fig.  118. 


Fig.   IIU. 


Fig.  120 — Retina  Two  Weeks 
after  Spontaneous  Detachment. 
Section  at  Macula.  Edema  of 
Retina.  Rod  and  Cone  Layer 
Well  Preserved. 


Fig.   118 — Old,    Degenerated    Detachment. 


Fig.  119 — The  same,  showing  Cellular  and 
Connective  Tissue  Degeneration. 


Fig.  120. 


DETACHMENT    OF    THE    RETINA.  177 

choroid  passes  through  the  fissures,  causing  proliferation  of  the  pigmented 
epithelium  with  fusion  and  scaring  of  retina  and  choroid.  Choroidal  and 
retinal  adhesio'is  tend  to  pre\'ent  detachment,  but  Hortsman  has  seen  sep- 
aration occur  in  such  cases.  Elschnig  considers  these  points  of  adhesion  as 
the  origin  of  the  rents  that  occur  in  detached  retinas.  7^he  hole  is  pro- 
duced bv  tearing  out  a  piece  of  adherent  retina  which  remains  attached 
to  the  choroid  when  separation  occurs.  Treacher  Collins  has  micro- 
scopically examined  two  cases  in  which  the  exact  condition  described  by 
Flschnig  existed.  He  also  calls  attention  to  the  common  picture  of  a  retina 
detached  except  at  one  or  two  places  which  are  regarded  as  points  of 
adhesion  that  later  may  break  away,  leaving  a  hole  in  the  retina.  Leber 
considers  the  inrolling  edges  of  a  retinal  laceration  as  evidence  of  \'itreous 
traction,  but  the  natural  tendency  of  a  torn  retina  is  to  curl  inward.  El- 
schnig saw  a  case  of  retinal  tear  in  which  inrolling  was  prevented  by  a 
retinal  vessel  which  crossed  the  opening.  Schrenberg  examined  a  case  of 
detachment  occurring  in  the  albuminuria  of  pregnancy.  He  found  extensive 
exudation  between  the  retina  and  choroid  and  thrombosis  of  many  choroidal 
vessels.  Much  has  been  written  about  exudation  from  the  choroid  as  a 
cause  of  detachment,  but  the  choroid  also  possesses  the  power  of  ab- 
sorption, and  it  is  possible  that  removal  of  subretinal  fluid  by  the  choroid 
may  assist  in  lowering  intraocular  tension  in  old  serous  detachment. 
Schwalbe  describes  a  lymph  space  between  the  pigmented  epithelium  and 
the  layer  of  rods  and  cones  which  may  be  of  importance  in  detachment. 
Tn  large  detachments  the  subretinal  fluid  is  more  albuminous  than 
the  vitreous.  Tn  a  measure,  this  may  be  due  to  its  isolated  situation  where 
loss  of  fluid  is  not  easily  replenished  from  the  ciliary  body. 

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Nordenson,  Die  Netzhautablosung. 
Parsons,   Oph.  Rev.,   1905,   XXIV,  161. 

Raehlmann,  A.   f.  O..  XXII,  4;    A.   f.  A.  XXVII.  1793,  p.  1. 
Rava    Annali  di  Otalmologie,  IX. 

Retzius,  Uber  d.   Baudes  Glaskorpers,  etc.,  Bd.   1;  Untersuchungen,  Bd.  VI,  1894,  67. 
Samelsohn.   Eericht  u.  d.    Heidel.   Oph.  \'arsaml.   1882;     Kl.    IMed.   f.  A.   1882. 
Schirmer,  R.   Ki.  M.  f.  A.,  IX.  1871.  246. 

Schmidt-Rimpler,  cited  by  Schobl,  Norris  &  Oliver,  III,  p.  533. 
Schijbl,    Xorris  &   Oliver,    III,   543  545. 
Schrenberg,   Kl.   Al.   f.   A.   1905,   S.  31. 
Spicer.  T.   O    S..  1900,  96. 
Uthoff,  Trans.  15  Int.  Med.  Cong.  1906:    A.  of  Oph.  1906,  535;    Heid.  Oph.  Soc.  Sept 

1903;    A.   of   Oph.,  XXXIII,   1904.   157. 
Von  Graefe,  A.  f.  O.  Bd.  1.  361-358  :   Bericht   Kl.   Med.  f.  A.  1886. 
Von  Hippel,  Oph.  Soc.  Heid.,  Arch,  of  Oph.,  XXXVIII,  60. 
VVagenmann,  A.  f.  O.,  XV,  209. 
Weeks,  Diseases  of  the  Eye,  1910. 
Wicherkewicz,    Rev.    gen.    d'Oph.,    1900,    546. 


Chapter  IX. 

DEGENERATIONS  OF  THE   RETINA. 

Retinitis  Pigmentosa  (Pigmentary  Degeneration  of  the  Retina). 
This  disease  is  a  non-inflammatory,  progressive  atrophy  of  the  entire 
retina,  attended  by  immigration  inward  of  cells  and  pigment  from  the 
layer  of  tessellated  epithelium.  I'he  clinical  symptoms  are  remarkably 
uniform  and  characteristic,  the  great  majority  of  cases  being  typical.  The 
changes  observed  with  the  microscope  are  wholly  degenerative.  In  well 
developed  cases  an  anterior  zone  of  the  retina  is  covered  with  a  network,  of 
jet-black  pigment.  The  pigment  deposits  consist  of  small  branching  lines 
which  form  figures  that  have  been  compared  to  bone  corpuscles  or  Haver- 
sian canal  systems.  Generally,  the  deposits  follow  the  course  of  retinal  ves- 
sels or  where  vessels  once  existed.  The  pigment  often  lies  in  front  of  the 
vessels,  proving  that  it  is  situated  in  the  inner  layers  of  the  retina.  Excep- 
tionally, it  tends  to  clump,  but  in  nearly  all  cases  a  few  branched  deposits 
also  are  present.  The  pigment  is  never  connected  with  white  patches,  hem- 
orrhages or  exudates.  Toward  the  disc  the  deposits  become  fewer  and 
fewer  until  they  cease,  leaving  an  unpigmented  region  around  the  nerve  and 
macula.  In  the  latest  stages  of  the  disease  these  regions  also  become  pig- 
mented. The  amount  of  pigmentation  varies  widely  in  different  cases.  It 
may  form  a  close  meshwork  or  be  limited  to  a  few  scattered,  spider-like  fig- 
ures; in  rare  cases,  it  may  be  lacking.  The  disc  assumes  the  dirty-yellow  cast 
of  retinitic  atrophy.  In  old  cases  scattered  colloid  dots  (drusen)  may  de- 
velop in  the  retina.  As  the  atrophic  process  advances,  the  larger  retinal  ves- 
sels become  greatly  narrowed  and  can  no  longer  be  traced  to  the  periphery, 
while  the  smaller  branches  become  invisible.  Shrinking  of  the  vessels  pro- 
ceeds uniformly,  differing  in  this  respect  from  the  irregularly  distributed  de- 
generation of  arteriosclerosis.  Rarely  do  the  vessels  exhibit  white  borders 
or  lines,  angiosclerotic  changes  being  conspicuously  absent.  Pigmentation 
commences  in  a  zone  just  anterior  to  the  equator  in  the  region  where  the 
posterior  and  recurrent  ciliary  arteries  of  the  choroid  anastomose.  From 
this  zone  the  area  of  pigmentation  extends  backward  toward  the  posterior 

179 


180  THE    FUNDUS    OCULI. 

pole  and  forward  toward  the  ora  serrata.  It  has  been  stated  that  pig- 
mentation Is  most  abundant  on  the  temporal  side  of  the  fundus. 

Both  eyes  are  affected  in  all  cases.  As  a  rule,  the  disease  is  con- 
genital or  commences  in  early  childhood.  The  first  and  most  conspicuous 
subjectiv^e  symptom  is  night  blindness  (hemeralopia)  which  exists  long 
before  contraction  of  the  field  can  be  demonstrated.  The  usual  history  is, 
that  from  childhood  the  patient  has  seen  little  or  nothing  at  night  or  in 
a  dim  light,  but  has  had  perfect  vision  in  full  daylight.  This  indicates  a 
torpid  state  of  the  retina.  All  perception  of  light  is  lost  first  in  the  anterfor 
zone  of  disease,  the  blind  belt  slowly  widening  toward  both  center  and  per- 
iphery. Vision  becomes  more  and  more  restricted  to  the  central  field,  and 
in  course  of  time,  the  patient  sees  objects  as  though  looking  through  a  tube. 
Orientation  suffers,  and  although  the  patients  may  be  able  to  read  fine 
print,  they  are  unable  to  walk  abroad  without  a  guide.  Early  in  the 
disease,  the  visual  fields  may  appear  normal  in  a  strong  light,  but  when  il- 
lumination is  reduced  shrinking  of  the  field  and  defective  vision  are  shown. 
In  typical  cases  blindness  commences  with  ring  scotoma,  although  a  strong 
light  may  be  necessary  to  demonstrate  it  (Fig.  121).  Extension  of  the 
scotoma  may  not  be  uniform  and  the  result  will  be  crescentic  sections  of  a 
ring  scotoma.  In  other  cases,  from  the  first,  the  fields  present  concentric, 
peripheral  contraction  (Fig.  122).  It  will  be  noticed  that  both  scotoma 
and  pigmentation  begin  in  the  same  zone  and  extend  in  a  similar  manner, 
but  the  amount  of  pigmentation  bears  no  relation  to  the  degree  of  retinal 
atrophy  or  loss  of  function.  No  particular  change  occurs  in  the  color 
sense.  Sometimes  the  fields  for  colors  contract  before  that  for  white,  but 
in  most  cases  the  two  contract  simultaneously  and  maintain  their  normal 
relations.  Photopsia?  are  very  rare.  Leber,  among  54  cases  of  retinitis 
pigmentosa,  saw  but  one  which  complained  of  sparks  and  flashes  of  light. 
The  presence  of  photopsia?  raises  the  presumption  that  the  case  is  one 
of  chorioretinitis  rather  than  retinitis  pigmentosa.  In  some,  but  not  all 
cases,  dispersion  of  retinal  pigment  leads  to  exposure  of  the  choroidal 
vessels.  In  this  disease  the  choroid  is  atrophic  and  the  pigment  accumulates 
In   the   Intervascular   spaces. 

Stereogram  53.  Retinitis  Pigmentosa.  Retinitic  Optic  At- 
rophy.* Right  eyeground  of  a  very  dark  complexioned  woman,  38  years 
of  age.  Family  history  unreliable.  Both  eyes  equally  affected.  Has  never 
been  able  to  see  in  a  dim  light.     Concentric  contraction  of  the  visual  fields 


From  German  Poliklinik.     Service  of  Dr.  H.  W.  Wootton. 


Fig.  121. 


lvcllvv*.lv.T   ?i<^»vS.v\.Vv>cv 


Fig.  122. 


DEGEXRR  \riOXS    OF    THi:    RKTIXA.  181 

progressively  advanced,  but  fair  central  vision  was  retained  until  three 
years  ago  when  that  also  failed.  At  the  present  time  she  has  bare  percep- 
tion of  light.  O plit lull niosco pic  Picture.  Probably,  this  fundus  was  once 
very  dark.  The  posterior  pole  still  retains  sufficient  pigment  to  hide  the 
choroidal  vessels,  but  in  the  equatorial  and  anterior  zones  the  choroid  is 
exposed.  F!xcept  immediately  around  the  nerve,  the  retina  is  covered 
with  large,  branching,  pigment  figures  of  the  peculiar  "  bone  corpuscle  " 
form  found  in  retinitis  pigmentosa.  The  pigment  figures  lie  in  front  of 
the  retinal  vessels  when  the  two  are  in  contact.  In  addition  to  the  pigment 
deposits,  the  retina  contains  a  number  of  scattered,  yellowish  dots,  sup- 
posed to  be  identical  with  the  colloid  bodies  or  drusen  shown  in  stereogram 
27.  1  he  main  retinal  vessels  are  greatly  narrowed,  while  the  smaller 
branches  have  wholly  disappeared.  1  he  optic  disc  presents  the  peculiar 
yellowish-grey  discoloration  characteristic  of  atrophy  secondary  to  degener- 
ation of  the  retina.  The  disc  is  rather  "  hazy  "  and  the  markings  of  the 
lamina  cribrosa   are  in\'isible. 

Dl.^GNOSis.  The  fundus  picture  is  that  of  retinal  atrophy.  That 
this  is  due  to  retinitis  pigmentosa  is  indicated  by  the  history  of  night 
blindness  commencing  in  childhood  and  progressive,  concentric  contraction 
of  the  fields,  terminating  in  blindness.  Chorioretinitis  is  excluded  by  the 
absence  of  white  fundus  patches  or  other  evidence  of  inflammatory  exu- 
dation and  also  by  the  symmetrical  and  uniform  distribution  of  the  lesions. 

Atypical  Forms.  Retinitis  Pigmentosa  Sine  Pigmento.  This 
is  a  rare  form  of  retinal  atrophy  with  the  history  and  course  of  retinitis 
pigmentosa.  There  is  night  blindness,  contraction  of  the  visual  fields, 
atrophy  of  the  optic  nerve  and  narrowing  of  the  vessels,  but  the  disease 
is  unaccompanied  by  pigmentation  of  the  fundus. 

Ri:tinitis  Punctata  Albescens.  In  this  form  of  retinal  atrophy 
the  fundus  is  studded  with  white  dots  instead  of  pigment.  See  stereogram 
54. 

Atrophia  Gvr  \ta  Choroid.^:  et  Retin.^.  The  above  title  has  been 
applied  to  a  form  of  retinal  atrophy  described  bv  Jacobson  and  by  Colman 
Cutler  but  classified  and  named  by  Fuchs.  It  appears  to  be  a  disease 
closely  related  to  retinitis  pigmentosa,  in  which  choroidal  atrophy  is  unduly 
prominent.  In  addition  to  all  the  symptoms  of  retinitis  pigmentosa,  there 
is  extreme  atrophy  of  the  choroid  which  commences  in  the  form  of  round, 
discreet,  white  spots  distributed  throughout  the  posterior  hemisphere.  The 
choroidal  spots  spread  until  they  become  confluent,  but  before  fusion  is 


182  THE    FUNDUS    OCULI. 

complete  they  are  separated  from  each  other  by  strips  and  islands  of 
normal  choroid.  In  advanced  cases  only  a  narrow  ring  of  normal  choroid 
surrounds  the  disc,  outside  of  which  the  choroid  is  white  and  atrophic. 
The  retina  is  pigmented  and  atrophic,  but  the  choroid  is  prematurely  af- 
fected at  the  posterior  pole,  differing  in  this  regard  from  retinitis  pig- 
mentosa in  which  atrophy  occurs  synchronously  in  both  choroid  and  retina 
and  a  lifetime  may  elapse  before  the  central  field  is  invaded.  The  situation 
and  development  of  the  choroidal  degeneration  suggest  an  obliterating 
disease  of  the  posterior  ciliary  arteries. 

A  few  cases  have  been  reported  as  retinitis  pigmentosa  in  which 
pigmentation  commenced  around  the  disc  and  extended  toward  the  periph- 
ery.* All  these  cases  lacked  the  typical  features  of  retinitis  pigmentosa, 
namely,  family  history,  hemeralopia,  concentric  contraction  of  the  visual 
fields,  etc.  Therefore,  they  should  not  be  classed  as  retinitis  pigmentosa. 
The  same  criticism  applies  to  cases  with  central,  instead  of  peripheral 
scotoma.  If  in  genuine  retinitis  pigmentosa  central  scotoma  is  superadded 
to  peripheral  scotoma,  it  is  probable  that  cerebral  or  optic  nerve  compli- 
cations exist. t 

Certain  types  of  visual  fields  may  be  considered  legitimate  for  reti- 
nitis pigmentosa.  These  are:  (1)  Peripheric  ring  scotoma;  (2)  Concen- 
tric contraction;  (3)  Irregular,  concentric  contraction;  (4)  Concentric 
contraction  with  retention  of  peripheral  field.  Other  variations  in  the 
visual  fields  should  be  accepted  as  genuine  with  caution. [j:  A  few  cases 
have  been  reported  of  pigmentary  degeneration  of  the  retina  that  were 
affected  with  day  blindness  [nyctalopia)  instead  of  night  blindness,  the 
retina  being  hypersensitive  instead  of  torpid.  Such  a  condition  is  so  at 
variance  with  the  vast  majority  of  genuine  cases  that  the  diagnosis  must 
be    regarded    as    extremely    doubtful. 

The  following  form  of  congenital  blindness  has  been  described  by 
Mooren  and  also  by  Leber.  That  the  infant  is  blind  is  inferred  from  the 
presence  of  nystagmus.  During  the  first  six  months  of  life  no  changes  are 
seen  in  the  fundus.  Then  occurs  thinning  of  the  pigment  layer  and  fine 
black  specks  appear  in  the  retina.  Later,  the  fundus  presents  the  picture 
of  retinitis  pigmentosa. 


*Scimemi ;     Sichel ;     Knapp ;     Dujardin. 

fSchmidt. 

X  Mellinger  has  recorded  a  case  as  retinitis  pigmentosa  with  normal  fields,  but  the 
case  was  otherwise  atypical.  The  right  eye  was  blind  from  glaucoma  and  the  left  did  not 
show  atrophy  of  the  vessels. 


DEGENERATIONS    OF    THE    RETINA.  183 

Wllbrand  and  Saenger  quote  nine  cases  reported  as  unilateral  retinitis 
pigmentosa.  This  disease  is  so  generally  bilateral  and  its  course  in  the 
two  eyes  so  symmetrical,  that  cases  reported  as  confined  to  one  eye  should 
not  be  accepted  as  genuine.  In  considering  this  and  other  atypical  forms 
of  retinitis  pigmentosa,  it  should  be  borne  in  mind  that  this  disease  may  be 
simulated  so  closely  by  other  conditions,  especially  congenital  syphilis,  as 
to  be  almost  if  not  quite  indistinguishable.  A  doubtful  class  of  cases  are 
those  of  congenital  pigmentation  of  the  retina  without  impairment  of 
vision.*  It  is  evident  that  these  could  not  be  cases  of  retinal  atrophy, 
which  is  the  essential  feature  of  retinitis  pigmentosa. 

Retinitis  pigmentosa  is  not  especially  infrequent.  Rosenbaum  esti- 
mated the  ratio  as  1 .5  cases  per  1,000  of  general  eye  diseases.  Schobl  found 
ten  cases  among  11,556  eye  patients.  The  disease  exhibits  a  preference 
for  the  male  sex.  In  191  published  cases  there  were  138  males  and  53 
females.  The  Semitic  races  are  said  to  be  more  frequently  aiiected  than 
the  Aryans. 

Course  of  the  Disease.  In  the  great  majority  of  cases  pigmentary 
atrophy  of  the  retina  appears  first  in  childhood  and,  undoubtedly,  many 
cases  are  congenital.  The  disease  develops  simultaneously  and  uniformly 
in  both  eyes  and  its  progress  is  extremely  slow.  Central  vision  and  color 
sense  usually  remain  normal  in  a  good  light,  until  the  limit  of  the  visual 
field  approaches  the  15th  parallel,  when  both  fail.  This  usually  occurs 
between  the  40th  and  60th  years  when  hopeless  blindness  develops.  Cases 
are  reported  in  which  some  vision  has  been  retained  until  the  80th  year. 
In  others  blindness  has  ensued  at  an  early  age,  sometimes  in  the  18th  or 
20th  year. 

In  true  retinitis  pigmentosa  no  interruption  or  arrest  of  the  disease 
can  be  expected.  Cases  in  which  improvement  is  said  to  have  occurred  are 
too  unusual  and  remarkable  to  affect  the  unfavorable  prognosis. f  On  the 
other  hand,  the  course  of  chorioretinitis  sometimes  may  be  arrested,  and  as 
certain  cases  of  the  latter  disease  are  diflficult  to  differentiate  from  retinitis 
pigmentosa,  individual  cases  may  be  treated  in  the  hope  that  they  belong 
to  a  spurious  type. 

Complications.  Posterior  cortical  cataract  in  one  or  both  eyes 
frequently  complicates  retinitis  pigmentosa.  It  was  present  in  54  out  of 
182    cases   cited   by   Wilbrand   and    Saenger.      These    cataracts    advance 


*Juler. 

fScheiss — Gemusus  ;  Groisz. 


184  THE    FUNDUS    OCULI. 

very  slowly  or  remain  stationary.  Recent  literature  would  indicate  that 
glaucoma  was  an  infrequent  complication  of  pigmentary  atrophy  of  the 
retina.  About  20  cases  are  published  in  which  this  combination  was  sup- 
posed to  exist.  In  several  of  these  the  diagnosis  of  retinitis  pigmentosa  was 
not  clearly  established.  Glaucoma  is  a  common  disease  and  it  remains  to  be 
proven,  that  its  occurrence  with  pigmentary  atrophy  is  more  than  mere 
coincidence.  E.  von  Hippie  examined  a  case  of  glaucoma  in  which  the 
retina  was  pigmented.  The  pigment  did  not  come  from  the  retinal  epi- 
thelium, but  was  supposed  to  have  separated  from  the  ciliary  processes 
and  been  carried  by  leucocytes  to  the  retina.  Both  Schweigger  and  Parsons 
have  seen  a  retina  pigmented  in  this  manner  and  Knape  saw  a  retina  which 
became  pigmented  after  detachment. 

Etiology.  The  most  potent  etiologic  factor  is  heredity.  Frequently 
several  children  of  the  same  parents  (siblings)  are  affected.  It  follows 
both  by  direct  and  indirect  descent.  Descent  may  be  intermittent  and  the 
disease  may  reappear  after  remaining  latent  for  three  or  four  generations. 
Blood  relationship  of  parents  appears  to  be  of  etiologic  importance, 
although  its  influence  is  denied  by  some  authors.  MacNamara  affirms 
that  in  India  where  religion  successfully  prohibits  marriage  between  rela- 
tives, retinitis  pigmentosa  is  as  frequent  as  elsewhere.  Nevertheless,  it 
is  a  matter  of  common  observation  that  in  many  cases  parental  consan- 
guinity exists.  Both  Liebreich  and  Beyer  found  it  in  one  half  of  their 
cases,  but  this  percentage  is  too  high,  subsequent  statistics  having  shown 
the  proportion  to  be  about  25  per  cent.  The  subject  of  heredity  in  this 
and  other  diseases  has  been  elaborated  by  Nettleship  and  he  leaves  the 
question  undecided,  but  states  that  it  is  of  importance  in  retinitis  pig- 
mentosa only  when  both  parents  are  of  tainted  stock.  Among  976  fam- 
ilies, including  1681  persons  with  retinitis  pigmentosa,  he  found  heredity 
without  consanguinity  in  23.5  per  cent.  Consanguinity  without  heredity  in 
23  per  cent;  and  combined  heredity  and  consanguinity  in  3  per  cent.  Syph- 
ilis has  been  regarded  by  many  as  a  common  cause  of  retinitis  pig- 
mentosa, and  Bocchi  states  that  it  usually,  if  not  always,  ciepends  upon 
syphilis  of  two  or  three  generations  back.  This  view  is  regarded  as 
erroneous,  the  error  being  due  to  the  close  resemblance  between  chorio- 
retinitis pigmentosa  (Stergm.  22)  and  retinitis  pigmentosa.  Schon  saw 
seven  cases  of  retinitis  pigmentosa  of  which  four  were  rachitic,  truly 
an  exceptional  experience.  Hemeralopia,  with  and  without  pigmentation 
of  the  retina,  has  been  observed  in  connection  with  chronic  diseases  of 
the  liver.    Landolt  considered  that  an  intimate  relationship  existed  between 


DEGENERATIONS    OF    THE    RETINA.  185 

hepatic  cirrhosis  and  diseases  of  the  choroid  and  retina,  a  theory  which 
has  not  been  proven.  Pigmentation  closely  resembling  that  which  occurs  in 
retinitis  pigmentosa  is  often  seen  in  eyes  blinded  by  injuries,  especially 
when  the  eye  contains  a  foreign  body  CFig.  127).  Despite  the  most 
careful  inquiry,  a  large  number  of  cases  remain  for  which  no  present  or 
remote  cause  for  the  disease  can  be  discovered.  This  was  true  in  six 
out  of  22  cases  reported  by  Webster,  which  is  about  the  usual  ratio.  True 
pigmentary  atrophy  of  the  retina  may  not  be  a  disease,  but  a  congenital 
structural  defect.  This  view  is  strongly  supported  by  its  frequent  associa- 
tion with  imperfections  in  the  nervous  system  and  malformations  of  the 
body.  The  most  frequently  associated  nervous  defects  are  imbecility  and 
idiocy  which  exist  in  about  10  per  cent  of  all  cases.  Deafmutism  and 
deafness  are  very  common.  Leibreich  in  38  cases  of  retinitis  pigmentosa 
found  14  deaf  mutes,  and  among  241  deaf  mutes  he  found  14  with  retinitis 
pigmentosa.  These  percentages  are  too  high,  Webster's  ratio  of  2  cases 
of  deafmutism  among  22  of  pigmentary  atrophy  being  more  accurate.  A 
larger  proportion  are  deaf  but  not  mute.  Many  cases  are  associated  with 
epilepsy  and  arrested  body  development.  Nearly  all  the  ocular  malforma- 
tions have  been  reported  as  occurring  with  retinitis  pigmentosa,  namely, 
coloboma  of  the  iris,  lens  and  choroid;  microphthalmus,  keratoconus,  pto- 
sis, high  myopia,  subluxated  lens,  persistent  hyaloid  artery  and  manifest 
canal  of  Cloquet.  Colloid  masses  on  the  disc  (drusenbildung)  occur  in 
some  cases  of  retinitic  pigmentation.  Some  cases  exhibit  nystagmus  or 
strabismus.  1  he  most  frequent  coexisting  body  deformities  are  poly- 
dactilia,  harelip  and  clubbed  feet.  Other  malformations  are  not  unusual. 
It  is  possible  that  retinitis  may  be  a  local,  premature  senility;  in  other  words, 
the  life  period  of  the  retina  is  shorter  than  that  of  other  body  organs. 
Cases  which  exhibit  vitreous  opacities,  interstitial  keratitis  or  iritis  should 
be  regardeci  as  either  syphilitic  or  tubercular. 

Diagnosis.  In  typical  cases,  the  family  history,  course  of  disease  and 
ophthalmoscopic  picture  of  retinitis  pigmentosa  are  unmistakable.  Atypical 
anci  doubtful  cases  are  the  exception.  Cases  which  present  all  features  of 
the  disease  except  pigmentation  or  those  in  which  white  dots  occur  instead 
of  pigment,  are  accepted  as  aberrant  types  of  retinitis  pigmentosa.  The 
presence  of  dav  blindness  instead  of  night  blindness  tends  to  invalidate 
the  diagnosis.  The  most  frequent  error  in  diagnosis  occurs  in  chorio- 
retinitis with  secondary  pigmentation  of  the  retina.  The  formation  of  pig- 
ment spider  figures  in  the  retina  is  not  peculiar  to  retinitis  pigmentosa 
but  may  occur  whenever  the  tessellated  epithelium  is  displaced  or  suffers 


186  THE    FUNDUS    OCULI. 

from  lack  of  nutrition.  Forster  affirms  that  syphilis  may  produce  a 
symptom  complex  almost  indistinguishable  from  true  retinitis  pigmentosa. 
In  both  diseases  there  are  contracted  fields,  peripheral  pigmentation  and 
yellow  atrophy  of  the  nerv^e.  The  pigment  deposits,  however,  are  not  so 
fine  as  in  pigmentary  atrophy  and  they,  do  not  bear  any  relation  to  the 
course  of  the  vessels.  Such  cases  must  be  uncommon.  In  syphilis,  a 
careful  search  usually  reveals  either  deep  exudates  or  white  patches. 
Moreover,  the  pigmentation  is  apt  to  be  irregularly  distributed,  and  the 
visual  fields  are  irregular.  In  doubtful  cases  the  presence  of  syphilitic 
stigmata,  i.  e.,  notched  incisors,  saddle  nose,  labial  scars,  etc.,  would  be 
decisive  as  would  also  vitreous  opacities,  uveitis  and  interstitial  keratitis 
In  all  cases  advantage  should  be  taken  of  serodiagnosis.  Pigmentary 
atrophy  may  be  associated  with  high  myopia,  in  which  event  choroidal 
atrophies  due  to  myopia  may  legitimately  appear  in  connection  with  the 
picture  of  retinitis  pigmentosa. 

Anatomic  Character.  The  first  subjective  symptom  of  retinitis 
pigmentosa  is  hemeralopia,  and  the  most  conspicuous  ophthalmoscopic  fea- 
ture results  from  dispersion  of  the  pigmented  epithelium,  all  of  which  in- 
dicates profound  ciisturbance  in  the  outer  layers  of  the  retina.  From  the 
fact  that  these  layers  are  nourished  by  the  choroid  it  is  assumed  that  the 
disease,  primarily  is  an  atrophy  of  the  choriocapillaris  and  that  the  retinal 
changes  are  due  to  lack  of  nutrition,  fhis  theory  is  supported  by  micro- 
scopic examinations  which  show  extreme  degeneration  of  the  choroid. 
This  satisfactorily  explains  destruction  of  the  outer  retinal  layers.  The 
inner  retinal  layers,  however,  which  include  the  retinal  vessels,  are 
wholly  independent  of  the  choroid  and  something  more  than  disease 
of  the  choriocapillaris  is  required  to  explain  the  extreme  atrophy 
which  they  also  undergo.  True,  an  exudative  choroiditis  may  de- 
stroy all  layers  of  the  retina  but,  in  retinitis  pigmentosa,  hyperemia 
and  exudation  never  occur.  Neither  do  the  subjective  symptoms  ac- 
cord with  a  primary  choroiditis.  Thus,  night  blindness  exists  for 
years  before  ophthalmoscopic  or  microscopic  changes  appear  in  the 
macula.  Ginsberg  examined  microscopically  a  case  of  typical  retinitis 
pigmentosa  and  found  areas  of  intact  choriocapillaris  beneath  highly 
atrophic  retina  and  other  areas  of  atrophic  choroid  covered  by  in- 
tact pigmented  epithelium,  from  which  he  drew  the  conclusion  that  the 
disease  was  a  primary  affection  of  the  neuroepithelial  layer.  Stock,  in  a 
case  of  central  pigmentary  degeneration  of  the  retina  (not  retinitis  pig- 
mentosa),  obtained  the  eye   for  examination  an  hour  and  a  half  after 


Fig.  12o. 

Retinitis    Pigmentosa.     Flat    Preparation    Showing    Bone-corpuscle    Arrangement    of 

Pigment. 


Fig.  124. 

Retinitis  Pigmentosa.     There  is  Universal  x^trophy  of  the  Retina  except  in  the  Macular 
'  Region  where,  as  shown  in  the  section,  the  Different  Layers  are  Distinguishable. 


DEGENERATIONS    OF    THE    RETINA.  187 

death.  The  only  certain  pathologic  alteration  was  degeneration  of  the 
rods  and  cones  which  Stock  regarded  as  a  primary  affection.  It  is  difficult 
to  understand  how  an  affection  of  the  neuroepithelium  can  induce  the 
choroidal  atrophy  of  retinitis  pigmentosa.  Gonin  and  Nettleship  agree 
that  the  cause  of  the  disease  may  be  an  insufficient  supply  of  blood  to  the 
choroid,  connected  in  some  way  with  the  anastomoses  between  the  posterior 
and  recurrent  ciliary  arteries,  which  occur  in  that  zone  of  the  fundus  where 
retinitis  pigmentosa  commences.  Experiments  upon  animals  are  instructive 
but  indecisive  as  to  the  primary  seat  of  retinitis  pigmentosa.  Thus,  section 
of  the  optic  nerve  and  posterior  ciliary  arteries,  deprives  both  choroid 
and  retina  of  blood  and  is  followed  by  pigmentation  of  all  layers  of  the 
retina.  Wagenmann  demonstrated  that  no  pigmentation  followed  section 
of  onlv  the  nerve  and  retinal  vessels.  Kriickmann  carefully  divided  a  few 
posterior  ciliary  arteries,  expecting  to  shut  off  the  blood  from  circum- 
scribed regions  of  the  choroid,  but  migration  of  pigment  occurred  only 
where  the  external  limiting  membrane  was  ruptured.  Kriickmann's  ex- 
periments are  inconclusive,  owing  to  the  free  anastomoses  between  cho- 
roidal vessels. 

I  have  had  but  one  opportunity  to  microscopically  examine  an  eye 
affected  with  retinitis  pigmentosa.  The  patient  was  64  years  of  age  and 
totally  blind.  The  left  eye  was  attacked  by  ulcerative  keratitis  and  enu- 
cleated for  relief  of  pain.  As  the  microscopic  findings  correspond  in  all 
essential  features  with  the  examinations  made  by  others,  It  Is  presented 
in  lien  of  further  description.  The  anterior  section  of  the  eyeball  pre- 
sented the  usual  features  of  keratoiritis.  The  cornea  was  not  perforated 
externallv,  but  Descemet's  membrane  was  ruptured.  The  lens,  anterior 
to  the  equator,  appeared  normal.  At  the  posterior  pole  was  a  zone  pre- 
senting the  usual  features  of  cataractous  degeneration.  The  retina  was 
adherent  to  the  choroid  in  places  and  could  not  be  separated  without  tear- 
ing. Flat  preparations  showed  bone  corpuscle-shaped  deposits  of  pigment 
covering  all  parts  of  the  retina  except  around  the  nerve  and  macula 
(Fig.  123).  Atrophy  of  the  retina  was  universal,  and  few  normal  ele- 
ments remained,  although  In  the  macular  region  the  different  layers  were 
still  distinguishable  (Fig.  124).  Here,  the  nerve  fiber  and  ganglion  cell 
layers  (a)  were  thickened  and  consisted  largely  of  neuroglia  and  con- 
nective tissue.  The  inner  reticular  (b)  and  nuclear  (t)  layers  were  com- 
paratively well  preserved.  The  outer  reticulum  (d)  was  highly  atrophic 
and  the  outer  nuclei  (e)  were  reduced  to  a  single  cell  layer.  The  rods 
and  cones  were  not  distinguishable   as  such  but  were  replaced  by  fibers 


188  THE    FUNDUS    OCULI. 

and  young  connective  tissue  cells  (/).  The  layer  of  pigmented  epithelium 
{g)  was  irregularly  thickened  and  some  cells  were  loosened  but  there 
was  no  migration  of  pigment.  The  choroid  was  better  preserved  here 
than  in  other  regions.  In  the  remaining  parts  of  the  retina,  the  normal 
structure  was  replaced  by  a  network  of  fibrous  tissue  which  took  a 
yellow  color  with  Van  Giesen's  stain.  In  most  regions  this  tissue  formed 
a  mere  tangle.  The  pigmented  epithelium  was  irregularly  distributed  and 
many  cells  were  poorly  pigmented  (Fig.  ,125).  In  some  places  the  pig- 
ment had  disappeared  and  in  others  proliferated.  Pigmented  cells  and 
free  pigment  were  scattereei  through  the  sclerosed  retina.  The  blood 
vessels  were  ensheathed  in  pigment.  Their  walls  were  greatly  thickened 
and  many  were  converted  into  strands  of  connective  tissue.  The  pigment 
was  deposited  first  in  the  perivascular  sheaths  (Fig.  126)  but  as  the 
vessels  dwindled  away  it  became  incorporated  in  their  walls,  and  in  the 
last  stage  of  atrophy  nothing  remained  of  the  vessel  but  a  line  of  pigment. 
The  choroid  was  highly  atrophic,  in  most  places  reduced  to  a  single  layer  of 
sclerosed  vessels  and  in  others  to  only  a  layer  of  pigment.  The  choroidal 
pigment  was  massed  against  the  lamina  vitrea,  but  did  not  enter  the  retina. 
The  choriocapillaris  had  mostly  disappeared,  but  In  some  places  a  few 
small  vessels  remained  even  where  the  retina  was  highly  atrophic. 

After  severe  wounds  of  the  eye  or  when  the  eye  contains  a  foreign 
body,  the  retina  may  undergo  a  form  of  pigmentary  degeneration  closely 
resembling  retinitis  pigmentosa.  An  example  of  this  condition  Is  shown 
In  Figure  127.  The  retinal  changes  are  microscopically  Indistinguishable 
from  those  of  retinitis  pigmentosa,  but  the  choroid  Is  comparatively  nor- 
mal while  In  true  retinitis  pigmentosa  it   always  Is  degenerated. 

In  many  pathologic  reports  it  Is  stated  that  a  sharp  line  of  demarca- 
tion separates  the  affected  from  the  unaffected  parts.  Ginsberg  found  the 
macular  region  nearly  normal.  Capauner  believes  the  pigmented  epi- 
thelium may  develop  ameboid  movement,  and  he  regards  pigmentation 
of  the  retina  as  due  to  active  emigration  of  the  cells.  If  the  retinal  epithe- 
lium possesses  such  function  It  does  not  appear  to  be  exercised  In  retinitis 
pigmentosa,  Inasmuch  as  the  cells  appear  to  perish  and  part  with  pigment 
before  emigration.  Probably,  they  are  passively  transported  by  the  retinal 
currents  and  deposited  In  the  perivascular  sheaths.  Possibly,  the  pig- 
ment obstructs  the  lymph  spaces  and  Induces  atrophy  of  the  vessels.  I 
have  found  no  pathologic  report  of  a  case  In  which  the  retina  contained 
no  pigment,  but  in  a  case  examined  by  Poncet,  pigment  too  fine  to  be  oph- 


Fig.  125  —  Retinitis  Pigmen- 
tosa, Complete  Degeneration  of 
the  Retinal  Layers  with  Irregu- 
lar Distribution  of  the  Pigment 
Epithelium. 


Fig.  126 — Retinitis  Pigmentosa.  R,  re- 
tina; C,  choroid;  S,  sclera.  Pigment  in 
the  Perivascular  Sheaths  in  the  Retina. 
Choroid  Highly  Atrophic.  Its  Pigment 
doc^  not  Invade  the  Retina. 


FiR.   V2i\. 


Fig.  127 — Retinal  Changes  due 
to  Injury  and  Indistinguishable 
Microscopically  from  Retinitis 
Pigmentosa.  The  Choroid,  how^- 
ever,  is  Normal. 


DEGENERATIONS    OF    THE    RETINA.  189 

thalmoscopically  visible  was  demonstrated  with  the  microscope.     The  optic 
atrophy  corresponds  in  degree  with  that  which  exists  in  the  retina. 

Bibliography. 

Beyer,  Inaug.  Dissert.,  1872. 

Bocchi,  Annali  d.  Ottal.    XXIV,  Sup.  21. 

Capauner.   23   Versaml.   d.    Heid.   Oph.   Gesell,    1893,    43. 

Cutler,  Arch,  of  Oi)h.  XXIV,  1895,  334. 

Dujardin,  Clin.   Oph.   1894,   125. 

Forster.  Graefc-Saeniisch   llandh.,  Vll,  .\uf.   1,   191. 

Fuchs,  A.  of  Oph.  XXVII,  1898,  484,  trans,  from  XXXII.  A.  f.  O. 

Ginsberg,  K.  M.  f.  A.,  July  1908;  Cent.  f.  p.  A.,  1906,  12. 

Gonin,  A.  d'O.  CXX\^  101. 

Groisz,  ref.  lahrsber.  f.  Oph..  1885.  413. 

Jacobson,  K."  M.  f.  A.  1888,  202. 

Juler,  Oph.  Rev.  1902,  22. 

Knape,  A.  f.  A.  XLVIII,  1903. 

Knapp,  Trans.   Am.   Oph.   Soc.   1870.   120. 

Kruckmann,  A.  f.  O.  XLVII,  3,  1899:    XLVIII,  1-2,  1899. 

Landolt.  A.  f.  O..  XVIII,  1. 

Leber,  A.  f.  O..  XV,  3,  S.  1;    XVII 1,  2,  S.  325. 

Liebreich,   Deutsch.   Klin.   Med.,   18<)1,   «. 

MacNamara,  Dis.  of  the  Eve,  2  edition,  1872. 

Mellinger.  K.  M.  f.  A.   XXIX,  171. 

Mooren,    Oph.    Beobacht.,    1867,   S.   260. 

Nettleship,  Tr.  O.  Soc,  XXVII,  1907;    XXX,  1910. 

Parsons,  Path,  of  the  Eye,   II,  595. 

Poncet,  A.   d'Ocul.,  LXXIV,  2.34. 

Rosembaum,  Inaug.   Dissert.,  Kiel. 

Scheiss-Gemusus,   K.   M.   f.   A.,  1875. 

Schmidt.   Inaug.   Dissert.,   Bonn,   1890. 

Schon.  C.   f.  p.   A..  1898,  15. 

Schweigger.   A.   f.   O.,  .5.   1.   1859;    IX,   1,   1863. 

Schobl,   Norris  &  Oliver,   III,  p.   460. 

Scimemi.   Annal.   di   Ottal.,   XXVTTT,   69. 

Sichel,   C.   f.  p.   A.   Apl.   1877. 

Stock,  Klin.  M.  f.  A.,  XLVI,  1.  225. 

Von  Hippel,  A.  f.  O.,  XL,  1,  1894;  A,  f.  O.,  XLII,  4,  1896;  LII,  1,  1901. 

Wagenmann,  A.  f.  O.,  XXXVI,  4,  1890. 

Webster,   Tr.   Am.   Oph.    Soc,   1878.  2,   495. 

Wilbrand  u.  Saenger,  IV,  28;  III,  100. 

Retinitis  Punctata  Albescens.  Under  the  above  title  Mooren 
described  a  condition  in  which  the  fundus  is  covered  with  white  dots  and 
the  optic  disc  atrophic.  In  consequence  of  Mooren's  brief  description, 
the  disease  was  confused  with  all  sorts  of  punctate  conditions  of  the  fundus. 
Order  was  brought  out  of  this  confusion  by  Nettleship,  Gayet  and  Fuchs 
who  showed  that  in  addition  to  white  spots  in  the  fundus,  a  strongly  marked 
group  of  clinical  symptoms  usually  existed.  As  now  understood,  retinitis 
punctata  albescens  is  a  progressive  atrophy  of  the  retina  presenting  all 
the  symptoms  of  retinitis  pigmentosa,  except  that  the  fundus  is  covered 
with  white  dots  instead  of  pigment.      In   Fuchs'  case,   the   smallest  dots 


190  THE    FUNDUS    OCULI. 

were  scarcely  distinguishable,  while  the  largest  did  not  exceed  the  diameter 
of  a  medium-sized  retinal  vessel.  They  were  white,  sharply  outlined,  and 
not  bordered  by  pigment.  Some  were  gathered  into  groups  or  rows.  The 
macular  region  and  periphery  of  the   fundus  were   free   from  dots. 

The  disease  prevails  in  certain  families,  and  parental  consanguinity 
frequently  exists.  The  first  subjective  symptom  is  night  blindness.  Con- 
centric progressive  contraction  of  the  visual  fields  commences  early.  As 
the  disease  advances  optic  atrophy  and  narrowing  of  the  retinal  vessels 
become  apparent.  The  disease  progresses  very  slowly  and  some  cases  ap- 
pear to  remain  stationary.  The  prognosis  Is  much  better  than  in  developed 
pigmentary  degeneration.  So  far  as  known  the  etiology  and  pathology 
are  those  of  retinitis  pigmentosa.  Pigment  may  be  present  in  the  retina 
in  specks  too  small  to  be  visible  with  the  ophthalmoscope  as  Poncet  found 
In  a  case  which  he  microscopically  examined.  The  white  spots  are  sup- 
posed to  be  deposited  by  the  retinal  epithelium  which,  Instead  of  parting 
with  pigment,  has  undergone  the  peculiar  proliferative  process  by  which 
colloids  (drusen)  are  formed  (Stergm.  27).  A  few  of  these  spots  are 
usually  present  In  advanced  cases  of  retinitis  pigmentosa    (Stergm.  S3). 

Stereogram  54.  Retinitis  Punctata  Albescens.*  Left  fundus 
oculi  of  a  man  36  years  of  age.  Urine  normal.  Has  been  unable  to  see 
in  a  dim  light  since  childhood.  His  father,  one  brother  and  two  sisters 
are  similarly  affected.  The  fundus  conditions  are  the  same  In  both  eyes. 
The  visual  fields  are  concentrically  contracted  (Fig.  128).  In  daylight, 
central  vision  Is  20/30.  Ophthalmoscopk  Picture.  Except  at  the  macula, 
the  fundus  Is  covered  by  groups  of  small  white  dots,  varying  In  size  from 
barely  visible  points  to  the  diameter  of  a  primary  retinal  vessel.  The 
dots  are  not  bordered  -by  pigment  but  a  few  independent  groups  of  fine 
pigment  specks  are  scattered  over  the  fundus.  .The  optic  disc  Is  atrophic. 
The  retinal  vessels  are  uniformly  narrowed.  The  arteries  are  rather 
tortuous,  but  this  Is  regarded  as  congenital  and  dependent  upon  an  exist- 
ing hypermetropla. 

Although  this  retina  is  not  entirely  free  from  pigment  the  pre- 
ponderance of  the  white  dots  entitles  the  case  to  be  classed  as  retinitis 
punctata  albescens  rather  than  retinitis  pigmentosa.  This  case  Is  especially 
valuable   as   showing  the   identity  of  these   two  conditions. 

Diagnosis.  Retinitis  punctata  albescens  requires  to  be  differentiated 
from  the  following  punctate  conditions.  Senile  colloids  and  Gunn's  dots 
are  limited  to  the  posterior  pole  or  macular  region.     The  lesions  of  cho- 

*  This  patient  was  seen  through  the  courtesy  of  Dr.  H.  H.  Tyson. 


DEGENERATIONS    OF    THE    RETINA.  191 

roiditis  are  never  so  small  as  those  of  punctata  albescens.  Central  punctate 
diabetic  retinitis  is  distinguished  by  the  size  of  the  spots  and  presence  of 
hemorrhage.  From  all  the  above  conditions  punctata  albescens  is  dif- 
ferentiated by  hemeralopia,  contracted  fields  and  ophthalmoscopic  appear- 
ance of  retinal  atrophy. 

Bibliography. 

Gavet.  Arch.  d'Oph.  Ill,  p.  385. 
Xettleship,  Oph.  Rev.   1887,  p.  181. 
Fuchs,  Arch.  f.  Augke.,  XXXIX. 
Poncet,  Arch.  d'Ocul.,  LXXIV,  p.  234. 

Maculocerebral  Degeneration  (Familial).*  A  few  peculiar 
cases  have  been  reported  which  present  a  symptom-complex  sufficiently 
uniform  and  distinct  to  require  independent  classification.  The  one  con- 
stant, characteristic  manifestation  is  a  degenerative  process  in  the  retinal 
macula  frequently  associated  with  a  similar  process  in  the  brain.  For 
descriptive  purposes,  the  affection  will  be  divided  into  (1),  the  maculo- 
cerebral type,  in  which  both  retina  and  brain  are  attacked,  and  (2),  the 
macular  type,  in  which  the  pathologic  changes  are  limited  to  the  retina. 

In  the  maculocerebral  type  the  patient  appears  perfectly  normal  until 
the  second  dentition,  that  is,  six  or  seven  years  of  age,  at  which  time 
simultaneous  failure  of  vision  and  intellect  ensues.  Invariably,  both 
eyes  are  affected.  In  developed  cases  the  ophthalmoscopic  picture  consists 
of  atrophy  and  pigmentation  of  the  retina  in  the  macular  region,  bleaching 
of  the  optic  nerve,  and  narrowing  of  the  retinal  vessels.  Small  light 
spots,  which  do  not  present  the  appearance  of  exudates,  may  exist  in 
the  surrounding  retina.  Neither  retinal  hemorrhage  nor  edema  has  been 
described.  The  earliest  ophthalmoscopic  change  is  slight  pigment  dis- 
turbance in  the  retinal  epithelium,  which  might  be  regarded  as  within 
normal  limits.  Even  this  change  may  be  preceded  by  failure  of  visual 
acuity.t  The  functional  eye  disturbance  consists  of  central  scotoma  for 
green  aild  red,  failure  of  central  vision  and,  sometimes,  day  blindness.  As 
the  disease  advances  the  scotoma  for  colors  increases  in  size,  but  does 
not  extend  to  the  periphery.  In  the  late  stages  the  scotoma  becomes  ab- 
solute, and  central  perception  of  light  is  lost.  Apparently  the  degenerative 
process  does  not  extend  to  the  anterior  zone  of  the  retina,  and  the  per- 
ipheral field  retains  its  normal  boundary,  vision  and  color  sense.  As 
central  vision  disappears,  the  patient  develops  eccentric  fixation,  attended 

'Oatman,    American    Journal   of    The    ^ledical    Sciences.    Aug.    1911 ;     Arch,    of    Oph., 
XXXVI.  1907,  p.  554, 
tSturgardl. 


192  THE    FUNDUS    OCULI. 

by  nystagmus  and  lateral  deviation  of  eyes  and  head.  Both  nystagmus  and 
lateral  deviation  may  diminish  or  disappear  should  the  subject  become 
completely  imbecile  and  no  longer  endeavor  to  use  the  eyes.  The  failure  of 
intellect  varies  from  irritability  to  imbecility.  In  some  cases  epileptiform 
convulsions  ensue.  From  the  few  cases  reported,  it  would  appear  that  the 
retinal  atrophy  commences  in  the  macula  and  progresses  rapidly  for  two 
or  three  years,  after  which  it  extends  very  slowly.  In  the  brain  the  disease 
pursues  a  similar  course,  and  after  the  intellect  has  sunk  to  the  grade  of 
medium  imbecility  its  progress  is  retarded.  No  case  has  become  com- 
pletely blind,  but  some  have  become  abject  imbeciles.  In  some  cases  the 
cerebral  degeneration  appears  to  extend  to  a  vital  center  and  cause  death. 

In  the  maculcu-  type  of  the  disease  the  characteristic  degeneration 
occurs  in  the  macular  region,  but  the  intellect  is  not  noticeably  affected.  It 
develops  later  than  the  maculocerebral  type,  usually  during  puberty,  when 
the  patient  is  from  fourteen  to  sixteen  years  of  age.  Reports  also  indicate 
that  in  the  macular  type  the  pupils  are  more  active,  and  the  nerve  does 
not  bleach  so  early  as  when  the  brain  is  involved.  None  of  the  patients 
were  epileptics. 

In  both  types  of  the  disease  all  the  subjects  have  been  reported  other- 
wise in  good  health  and  free  from  malformations.  In  every  instance  the 
affection  was  confined  to  one  or  more  members  of  a  childship,*  and  not 
found  among  ancestors  or  descendents.  Diseases  which  occur  in  this  man- 
ner are  termed  '*  familial."!     No  case  has  been  reported  among  Hebrews. 

The  following  two  cases  were  typical  examples  of  the  maculo- 
cerebral type.  The  childship  consists  of  three  children,  of  whom  the  eldest 
and  youngest  are  affected,  while  the  intermediate  child,  a  girl  aged  ten 
years,  is  intelligent  and  normal  in  all  respects.  The  parents  are  not  related 
by  blood,  but  there  is  an  indefinite  historv  of  intermarriages  among 
ancestors. 

Stereogram  S^.  Maculocerebral  Degeneration.  Advanced 
Stage.  Girl  aged  twelve  years.  She  is  the  eldest  of  the  three  children 
mentioned  above.  She  possessed  good  vision  and  was  an  excellent  scholar 
until  the  age  of  seven  years,  when  vision  and  intellect  began  to  fail.  At 
the  age  of  nine  years  she  developed  epileptiform  seizures  which  have  con- 
tinued   at   irregular   intervals. 

Present  Condition.     Large,  robust  girl.     Repeated  Wassermann 


*  Childship :     All  the  children  of  the  same  father  and  mother. 

tFamilial   disease :     One  that  occurs  among  members  of   a   childship   but  has   shown 
little  or  no  tendency  to  affect  ancestors  or  descendents. 


L.E. 


R.E. 


\( fu uto   .\t  v,ctat«.  .Atti-J U'M. . 


^     Fig.  128.       11. 


L.L. 


R.E. 


Fig.  129.     -^ 


DEGENERATIONS    OF    THE    RETINA.  193 

tests  for  syphilis  and  von  Pirquet's  test  for  tuberculosis  were  negative;  no 
malformations;  is  a  medium  imbecile  but  talks  well,  and  has  a  good  memory 
for  remote  events;  habits  cleanly;  myopic  (-2.  D.)  ;  pupils  sluggish  but 
otherwise  normal;  has  eccentric  fixation,  with  head  and  eyes  turned  to 
right;  lateral  nystagmus;  has  neither  night  nor  day  blindness.  There  is 
absolute  central  scotoma  in  both  eyes,  roughly  estimated  as  40^  in  the 
right  and  10  '  in  the  left  (Fig.  129).  Peripheral  fields  are  not  contracted, 
and  possess  good  perception  of  white  and  colors. 

Ophthalmoscopic  Picture.  Media  clear.  Strongly  marked 
tigered  brunette  fundus.  Optic  nerve  decidedly  atrophic.  Retinal  vessels 
narrowed  but  not  otherwise  changed.  The  smaller  vascular  twigs  have 
mostly  disappeared.  The  macular  region  exhibits  a  pigmentary  atrophy 
of  the  retina  limited  to  an  area  with  a  diameter  about  two  and  a  half  times 
that  of  the  optic  disc.  In  this  region  the  fundus  is  mottled,  has  a  dirty, 
yellowish-grey  color  and  is  peppered  with  granular  and  dust-like  pigment. 
A  number  of  ill-defined,  light  radiating  lines,  extend  outward  from  the 
temporal  side  of  the  nerve.  They  appear  to  be  light  reflexes.  Both  eyes 
are  affected  symmetrically.  In  the  right  eye  the  retina  is  more  atrophic, 
the  fundus  lighter  in  color  and  the  pigment  deposits  are  being  dissipated. 

Four  years  later:  The  patient  has  almost  reached  the  stage  of  com- 
plete imbecility.  Mem.ory  poor.  Saliva  collects  in  pharynx  and  interferes 
with  speech.  Peripheral  fields  not  contracted.  Can  distinguish  red  in  large 
mass.  Central  blind  area  does  not  appear  to  exceed  40 \  The  retinal  pig- 
ment as  far  as  the  equator  is  greatly  thinned  and  the  whoh  fu.-idus  is  much 
paler  than  four  years  ago.  The  central  area  is  veiled  as  though  the  retina 
were  converted  into  a  fibrinous  membrane.  In  the  macular  region  the 
pigment  has  entirely  disappeared  and  several  orange  colored  choroidal 
vessels  are  exposed. 

One  year  later  this  patient  died  with  symptoms  indicating  that  the  de- 
generative process  had  extended  to  some  vital  cerebral  center.* 

Stereogram  S6.  Maculocerebral  Degeneration;  Early 
Stage.  Right  eyeground  of  boy,  aged  eight  years.  Brother  of  case 
(Stergm.  55)  described  above.  Wassermann  reaction  for  syphilis  and 
von  Pirquet  for  tuberculosis,  negative.  Was  considered  a  normal  child 
until  six  years  of  age,  when  failure  was  noticed  in  vision  and  intellect.  He 
is  in  excellent  health;  no  paralysis;  no  malformations.     Vision  in  right  eye 


*  Unfortunately,   T    was   unable   to   secure   an    autopsy-. 


194  THE    FUNDUS    OCULI. 

is  10/200;  in  left  8/200.  Small  central  scotoma  for  green  and  red;  per- 
ipheral fields  normal  for  white  and  colors;  central  fixation;  talks  well  and 
memory  good. 

Ophthalmoscopic  Picture.  Media  clear.  The  macula  is  encir- 
cled by  a  transversely  oblong  ring  of  granular  pigment,  measuring  about 
one  disc  diameter  in  length.  The  enclosed  area  has  a  dirty,  yellowish  cast 
and  contains  dark  spots  which  can  be  resolved  into  fine  black  granules. 
The  surrounding  retina  is  covered  with  dust-like  pigment.  The  optic 
nerve  is  white  on  the  temporal  side.     Retinal  vessels,  narrowed. 

The  left  eye  presents  the  same  picture  as  the  right,  except  that  the 
macula  within  the  pigment  ring  is  more  atrophic  and  the  fundus  as  a 
whole,  somewhat  lighter  in  color. 

Four  years  later.  The  disease  has  steadily  advanced.  No  paralysis. 
Central  scotoma  is  now  absolute.  Peripheral  fields  appear  normal.  Mem- 
ory poor.  Is  apathetic  and  makes  little  effort  to  see.  Eccentric  fixation 
with  eyes  upward.  Retina  is  becoming  depigmented.  Has  had  five  epi- 
leptiform convulsions,  the  first  of  which  occurred  three  years  ago. 

The  intermediate  child  is  now  fourteen  years  of  age.  She  is  a  nor- 
mal, healthy  girl,  with  perfect  visual  acuity. 

Cases  of  maculocerebral  type  were  reported  first  by  F.  E.  Battin  of 
London,  in  1903  as  "  Cerebral  Degeneration,  with  Symmetrical  Changes 
in  the  Macula  in  Two  Members  of  a  Family."  Their  vision  and  mental 
condition  gradually  deteriorated,  and  they  were  removed  to  an  asylum 
where   eventually  they  died.     They  did  not  become   entirely  blind. 

M.  S.  Mayou  in  1904  reported  three  similar  cases  in  a  childship  of 
seven  children.  Parents  were  first  cousins.  In  her  first  pregnancy  the 
mother  miscarried  at  three  months.  The  second,  third  and  fourth  children 
were  born  healthy  but  developed  maculocerebral  degeneration,  while  the 
fifth,  sixth  and  seventh  were  both  healthy  and  intelligent. 

Six  years  later  Mr.  Mayou  courteously  writes  as  follows:  "  My  cases 
are  still  alive  and  the  eye  condition  remains  stationary.  The  eldest  child 
has. become  a  complete  imbecile,  and  the  other  two  children  are  in  schools 
for  the  mentally  defective.  The  mental  condition  appears  to  have  a  slowly 
progressive  tendency,  but  the  children  are  not  blind." 

Other  cases,  supposed  to  be  maculocerebral  degeneration  have  been 
recorded  by  Nettleship,  and  Hirschberg. 

Macular  Degeneration.  As  stated,  the  macular  type  presents 
the  history  and  every  clinical  feature  of  the  disease,  except  failure  of  in- 
tellect. The  period  of  development,  however,  is  delayed  until  the  age  of 
puberty. 


DKGEXEKATIOXS    OV     IHi:    RKTINA.  195 

Two  examples  described  by  R.  D.  Battin,  in  1897,  appear  to  be  the 
first  of  either  type  reported. 

Under  the  title  of  "  Family  Progressive  Degeneration  in  the  Macular 
Region  of  the  Eye,"  K.  Stargardt  has  published  the  history  of  two  child- 
ships  which  are  typical  examples  of  the  macular  type.  The  first  childship 
consisted  of  four  children,  all  of  whom  were  affected.  There  was  no 
family  history  of  eye  disease,  consanguinity  of  parents  or  syphilis.  Aside 
from  the  eye  affection,  the  patients  were  healthy,  free  from  malformations 
and  of  average   intelligence. 

Stargardt's  second  childship  consisted  of  five  children,  three  of  whom 
developed  macular  degeneration.  Except  the  macular  disease,  every  mem- 
ber of  the  childship  was  intelligent,  healthy  and  free  from  malformations. 

Stargardt  cites  a  further  case  from  Leber's  records  which  appears  to 
be  an  example  of  the  macular  type. 

Pathologic  Anatomy.  In  the  absence  of  a  microscopic  examination 
the  histologic  changes  can  be  surmised  only  from  the  ophthalmoscopic  pic- 
ture. This  indicates  an  atrophic  degeneration  of  the  retina,  commencing  in 
the  outer  layers.  The  earliest  visible  change  is  loss  of  foveal  reflex  and 
rarefaction  of  pigment.  The  loosened  pigment  is  next  distributed  through- 
out the  retina  as  a  fine  dust  or  specks  which  exhibit  little  tendency  to  wander 
inward  over  the  retinal  vessels.  The  pigment  does  not  proliferate,  neither 
is  it  fixed,  inasmuch  as  it  eventually  disappears.  Ultimately,  the  retina  is 
converted  into  a  thin  cicatricial  membrane,  constituting  the  delicate  film 
which  cov^ers  the  macular  region  in  advanced  cases.  When  depigmentation 
is  complete,  the  choroidal  vessels  are  exposed.  In  my  case,  in  which  the 
choroid  was  thus  revealed,  the  interspaces  contained  no  pigment,  although 
normally  this  was  a  strongly  "  tessellated  "  fundus.  Depigmentation  of 
the  fundus  oculi  may  be  due  either  to  local  action  of  some  adventitious 
substance,  or  the  pigment  granules  may  be  removed  by  leucocytes.*  The 
absence  of  all  evidence  of  inflammatory  action  suggests  that  the  retinal 
atrophy  results  from  nutritive  disturbance.  Inasmuch  as  the  retinal  layer 
first  affected  Is  nourished  entirely  by  the  choroid,  it  might  be  inferred  that 
the  primary  lesion  is  In  the  choriocapillarls.  Furthermore,  limitation  of 
the  pathologic  changes  to  the  posterior  pole  of  the  eye  corresponds  to  the 
distribution  of  a  choroidal  vascular  circuit.  Extreme  Interest  attaches  to 
the  work  of  Stock  (vide  infra) .  In  three  cases  of  primary  retinal  degenera- 
tion he  appears  to  have  demonstrated  by  microscopic  examinations  that 


*  I  have  seen  complete  depigmentation  of  the  eveground  in  a  diabetic   (Arch,  of  Oph. 
1910.   XXXIX,  p.  392.)      (Stergm.  26.) 


196  THE    FUNDUS    OCULI. 

the  primary  change  was  a  degeneration  of  the  rods  and  cones,  probably 
caused  by  selective  action  of  some  toxin  In  the  blood.*  No  case  reported 
Indicates  that  the  deep  layers  of  the  choroid  were  affected.  The  light 
spots  described  In  many  cases  should  be  regarded  as  degenerative  rather 
than  exudative.  They  may  have  been  due  either  to  exposure  and  thickening 
of  the  lamina  vltrea  or  to  condensation  and  thickening  of  the  retinal  neu- 
roglia. In  Stargardt's,  and  also  In  my  cases,  the  spots  were  not  prominent 
and  could  be  seen  with  the  ophthalmoscope  only  by  the  direct  method. 
Their  tendency  to  fade  as  the  light  was  moved  indicated  reflexes  rather 
than  actual  deposits.  Probably  the  optic  atrophy  Is  secondary  to  retinal 
degeneration,  although,  as  regards  cerebral  cases,  this  Is  not  certain.  In 
my  second  case  the  nerve  was  white  and  vessels  narrowed,  early  In  the 
disease.  In  the  macular  type,  however,  a  late  secondary  atrophy  Is 
described. 

Etiology.  The  records  throw  little  light  upon  the  cause  of  the 
affection.  In  one  chlldship  congenital  syphilis  was  surmised,  but  not  proved. 
Certainly,  It  did  not  exist  in  my  cases.  Racial  predisposition,  as  In  amau- 
rotic family  idiocy,  may  be  excluded,  as  none  of  the  cases  occurred  among 
Hebrews,  but  were  distributeci  among  various  other  races.  Hereditary  tend- 
encv  to  eye  diseases  did  not  prevail  in  any  of  the  affected  families.  Blood 
relationship  of  parents  may  be  measurably  causative;  thus,  it  existed  In 
four  of  the  six  chlldshlps  affected  with  the  maculocerebral  type,  and  in 
one  of  the  four  chlldshlps  affected  with  the  macular  tvpe.  The  fundus 
changes  are  not  unlike  those  which  occur  in  the  aged,  as  though  the  retina 
were  affected  with  a  local,  premature  senility.  Nettleship  suggested  that. 
a  cause  for  retinal  degeneration  in  children  might  be  found  In  nutritive 
disturbance  due  to  the  exanthemata  or  other  exhausting  conditions.  In  the 
pigmentary  atrophies  of  the  retina.  Stock  argues  that  an  autocytotoxin 
exists  in  the  blood,  which  exerts  a  selective  action  upon  the  rods  and  cones. 
Stargardt,  applying  this  theory  to  his  cases,  suggests  a  toxin  with  a  selective 
action  for  cones  only.  Dr.  Max  Schlapp,  who  observed  my  cases  also 
regarded  the  pathologic  changes  as  due  to  the  action  of  an  autotoxin  upon 
the  nervous  elements  of  the  brain  and  retina.  It  is  important  to  note  that 
the  maculocerebral  type  appears  with  second  dentition,  while  the  macular 
type  is  delayed  until  the  period  of  puberty.  It  Is  Improbable  that  this  Is 
mere  coincidence.  More  likely  the  developmental  forces  active  during 
dentition  and  sexual  evolution  are  here  perverted  to  stimulate  a  latent 
degenerative  tendency. 

*Possibly  syphilis. 


DEGENERATIONS    OF    THE    RETINA.  197 

DiFFERENTLATiON.  Several  affections  exist  which,  while  differing 
from  each  other,  all  bear  some  resemblance  to  maculocerebral  degener- 
ation. These  are,  hereditary  macular  anomaly,  retinitis  pigmentosa,  amau- 
rotic family  idiocy,  central  chorioretinitis,   and  certain  unclassified  cases. 

Hereditary  Macular  Anomaly.  This  condition  has  been  well 
presented  by  F.  Best,  who  described  the  remarkable  ophthalmologic  history 
of  a  family  of  whom  8  members  presented  a  defect  at  the  macula.  In 
all  other  respects,  however,  the  cases  differed  essentially  from  maculocere- 
bral degeneration.  The  family  was  traced  through  five  generations,  and 
59  individuals  were  examined,  of  whom  31  presented  some  affection  of 
the  eyes.*  The  8  affected  with  macular  anomalies  were  distributed  among 
5  childships.  The  defect  occurred  by  direct,  indirect  and  collateral  in- 
heritance.! In  6  of  the  cases  both  eyes  were  affected,  and  in  2  the  change 
was  rudimentarv  and  limited  to  one  eye.  In  every  case  the  defect  was  sit- 
uated immediately  below  the  point  of  fixation,  a  uniformity  which  Best 
regarded  as  a  remarkable  example  of  persistence  in  hereditary  transmission. 
Among  the  cases  with  macular  defect  the  following  additional  abnormal- 
ities were  found:  high  hypermetropia,  astigmatism,  strabismus,  persistent 
pupillary  membrane,  and  one  case  of  total  color  blindness.  None  of 
the  cases  had  epilepsy,  imbecility,  or  optic  atrophy.  Aside  from  the  one 
case  of  total  color  blindness,  the  color  sense  was  normal  in  all.  Visual 
acuity  was  blunted,  but  none  ot  the  subjects  was  incapacitated.  The  poor 
vision  had  existed  since  early  childhood,  and  was  non-progressive.  During 
the  eight  years  they-  were  under  observation  no  changes  occurred  in 
the  eyes.  From  these  facts  Best  justly  decided  that  the  condition  was  an 
hereditary  macular  anomaly. 

Maculocerebral  degeneration  differs  from  such  congenital  defects  in 
its  family  history,  late  development,  progressive  course,  cerebral  compli- 
cations, central  color  scotoma,  optic  atrophy  and  invariable  bilateral 
occurrence. 

Retinitis  Pigmentosa.  At  first  glance,  maculocerebral  degenera- 
tion appears  like  an  atypical  retinitis  pigmentosa.  Both  affections  develop 
in  early  life,  are  slowly  progressive,  and  present  a  symmetrical  picture  of 


*Macular  defects,  8 :  opaque  nerve  fibres,  1  :  one-sided  amblyopia,  5 ;  double  amblyopia 
with  nystagmus,  2;  strabismus  convergence,  3;  pigment  points  in  eyeground,  6:  senile 
cataracts,  5  ;  punctiform  opacities  at  posterior  pole,  6 ;  ptosis  and  telangiectasis  of  lid,  1 ; 
glioma,  1 :  hypermetropia,  7 ;  hyperopic  astygmatism,  8 ;  anisometropia,  7.  None  of  the 
cases  were  myopic. 

t  Direct  inheritance :  From  parent  to  child.  Indirect  inheritance:  From  grand- 
parents, uncles,  or  aunts.  Collateral  inheritance :  Occurrence  of  the  same  anomaly  among 
brothers  and  sisters ;    thus,  familial  disease  occurs  by  collateral  inheritance. 


198  THE    FUNDUS    OCULI. 

atrophy  in  each  retina.  Furthermore,  it  is  estimated  that  10  to  13  per 
cent  of  retinitis  pigmentosa  cases  are  idiots.  These  analogies,  however, 
are  more  than  counterbalanced  by  the  following  features  in  which  the  two 
diseases  differ.  The  most  constant  symptom  in  retinitis  pigmentosa  is 
night  blindness.  Supposititious  cases,  in  which  day  blindness  existed,  are  too 
few  in  number  to  vitiate  the  rule.  The  presence  of  night  blindness  more 
than  anything  else  has  caused  retinitis  punctata  albescens,  retinitis  pigmen- 
tosa sine  pigmento,  and  gyrate  atrophy  to  be  classed  as  aberrant  forms  of 
retinitis  pigmentosa.  Opposed  to  this  are  20  cases  of  maculocerebral  degen- 
eration, none  of  which  had  hemeralopia,  while  3  saw  better  in  a  dim  light. 
Retinitis  pigmentosa  is  eminently  hereditary  and  the  persistence  with 
which  it  reappears  in  families  renders  it  easy  to  trace  its  history.  In  mac- 
ulocerebral degeneration,  however,  the  family  trees  are  remarkably  exempt 
from  eye  diseases.  True  retinitis  pigmentosa  invariably  commences  near 
the  periphery  and  all  parts  of  the  retina  are  invaded,  the  macula  last  of 
all.*  Macular  degeneration,  on  the  contrary,  commences  in  the  macula,  and 
does  not  appear  to  extend  into  the  periphery.  In  retinitis  pigmentosa  the 
color  sense  fails  only  with  light  perception,  but  in  maculocerebral  degenera- 
tion one  of  the  earliest  manifestations  is  central  scotoma  for  green  and  red. 
The  idiocy  associated  with  retinitis  pigmentosa  always  is  congenital,  while 
the  subjects  of  maculocerebral  degeneration  are  born  with  normal  intellects, 
which  subsequently  may  be  destroyed  by  the  disease.  Malformations  of 
all  kinds,  so  common  in  retintis  pigmentosa,  are  lacking  in  maculocerebral 
degeneration.!  Finally,  retinitis  pigmentosa  is  transmitted  for  genera- 
tions, while  maculocerebral  degeneration  is  a  familial  disease. 

Amaurotic  Family  Idiocy.  Hirschberg  remarked  that  his  case 
presented  some  symptoms  not  unlike  amaurotic  idiocy,  but  differed  in  other 
and  essential  points.  Nettleship,  however,  is  disposed  to  trace  a  relation- 
ship between  the  two  diseases.  He  suggests  that  amaurotic  family  idiocy 
may  sometimes  pursue  a  mild  course  and  permit  the  child  to  grow  up,  and 
cited  the  cases  of  J.  D.  Battin  and  Mayou  as  possible  examples.  He 
avoided  the  racial  difficulty  on  the  supposition  that  Jewish  blood  flows  in 
manv  gentiles.  Apparently  the  two  diseases  are  entirely  distinct.  Amau- 
rotic idiocy  appears  to  occur  exclusively  among  Hebrews.  Cases  reported 
among  other  races  have  not  been  accepted  as  genuine.  The  lesion  is  a  pri- 
mary degeneration  of  the  ganglion  cells  throughout  the  nervous  system  in- 

_  *Cases  of  Knapp,  Dujardin,  Scimmi  and  Sichel,  reported  as  retinitis  pigmentosa  com- 
mencing at  tlie  macula,  are  lacking  in  other  essential  features  of  that  disease  and  cannot 
be  accented  as  genuine. 

trlirschberg's  cases  presented  stigmata,  not  deformities. 


DEGENERATIOXS    OF    THE    RETINA.  199 

eluding  those  of  the  retina.  The  disease  develops  a  few  months  after 
birth  and  eventuates  in  general  muscular  paresis,  idiocy,  and  blindness, 
the  patient  usually  dying  within  two  or  three  years.  In  contradistinction 
to  this  history,  maculocerebral  degeneration  develops  In  late  childhood, 
and,  so  far,  only  among  gentiles.  In  no  case  has  muscular  weak- 
ness, paralysis,,  or  ill  health  been  attributed  to  the  disease;  neither  has  any 
case  become  entirely  blind.  Anatomically,  the  diseases  differ  essentially. 
Macular  degeneration  begins  In  the  outer  layers  of  the  retina,  and  Is  limited 
to  the  central  region.  Amaurotic  Idiocy,  on  the  contrary,  begins  In  the 
inner  layers  and  extends  over  the  entire  retina.  By  no  means,  is  it  espe- 
cially a  macular  disease;  all  regions  of  the  retina  are  equally  affected,  but 
the  opacity  is  most  conspicuous  about  the  fovea  simply  because  here  the 
dead  ganglion  cells  are  eight  or  nine  layers  In  thickness  as  compared  with 
a  single  layer  elsewhere.  The  strong  ophthalmoscopic  picture  thus  pro- 
duced appears  to  have  engendered  an  erroneous  Idea  that  amaurotic  family 
idiocy  is  a  macular  disease.  Treacher  Collins,  who  saw  F.  D.  Battin's  cases, 
said  the  dark  spot  at  the  macula  was  composed  of  definite  pigment  granules, 
and  was  not  due  to  contrast  as  In  amaurotic  idiocy. 

Central  Chorioretinitis.  The  usual  form  of  central  choroiditis 
bears  little  resemblance  to  maculocerebral  degeneration.  Choroiditis  fre- 
quently depends  upon  evident  syphilis  or  tuberculosis.  It  presents  inflam- 
matory phenomena,  and  the  early  lesions  appear  as  deeply  situated  foci  of 
exudation.  These  are  followed  by  formation  of  white  cicatrices  and  mass- 
ing of  pigment.  The  history  and  course  of  maculocerebral  degeneration 
have  no  counterpart  In   exudative  choroiditis. 

Unclassififd  Cases.  Ophthalmic  literature  contains  many  unclassi- 
fied cases,  which  may  or  may  not  belong  to  the  same  group  as  maculo- 
cerebral degeneration.  The  most  important  are  the  following  by  Stock. 
Although  regarded  by  the  author  as  an  Independent  disease,  it  is  strongly 
suspected  that  they  were  cases  of  congenital  syphilis. 

Four  children  In  a  chlldship  were  affected.  No  parental  consan- 
guinity. After  the  birth  of  the  first  child,  which  was  healthv,  and  escaped 
the  disease,  the  father  acquired  syphilis  (statement  uncertain),  and  now  Is- 
in  America.  The  four  patients  were  normal  until  they  were  six  years  of 
age,  when  vision  and  Intellect  began  to  fall.  They  exhibited  posterior  iritic 
adhesions  and  multiple  glandular  enlargements.  The  Iritic  adhesions  dis- 
appeared after  mercurial  inunctions.  No  paralvsls.  One  case  was  epi- 
leptic. They  rapidly  became  absolute  imbeciles,  and  completely  blind. 
At  first  the  fundus  presented  no  visible  change,  but  later  pigmentation  of  the 


200  THE    FUNDUS    OCULI. 

retina  commenced  at  the  periphery  and  extended  toward  the  center.  The 
optic  nerv^e  and  retinal  arteries  showed  no  positive  evidence  of  atrophy. 
Three  of  the  cases  died  from  tuberculosis  and  the  eyes  were  secured  for 
examination,  in  one  case  an  hour  and  a  half  after  death.  The  findings  were 
of  the  first  importance  in  their  bearing  upon  all  forms  of  essential  retinal 
atrophy.  Optic  nerves  were  normal.  Choroid  and  inner  layers  of  the 
retina  were  comparatively  free  from  degeneration.  The  only  certain 
pathologic  change  was  more  or  less  complete  degeneration  of  the  rods 
and  cones.* 

This  disease  resembled  maculocerebral  degeneration  in  that  it  was 
aroused  to  activity  during  the  second  dentition  and  manifesteci  itself  by 
destruction  of  vision  and  intellect.  It  may  be  differentiated,  however,  by 
the  presence  of  other  eye  lesions,  constitutional  taint,  acute  course,  absence 
of  optic  atrophy,  and  primary  location  of  the  retinal  disease  at  the  per- 
iphery. The  limitation  of  maculocerebral  degeneration  to  the  central  zone 
Is  alone  sufiicient  to  separate  the  two  conditions. 

In  the  ophthalmoscopic  picture  of  maculocerebral  degeneration  neither 
the  amount  of  pigment  nor  its  arrangement  has  much  bearing  upon  diag- 
nosis. The  essential  point  is  the  presence  of  "  simple  "  as  distinguished 
from  postinflammatory  atrophy  of  the  retina  limited  to  the  posterior  pole. 

Bibliography. 

Battin.  F.  E.,  Transactions  Ophthalmological   Society,  1903,  XXIII,  386. 
Battin,  R.  T).,  Trans.  Ophthalmological   Soc.  United   Kingdom.   1897,  XVII,  48. 
Best.    An    Hereditary   Macular    Affection.   Zeits,   f.    Angenh.,   1905.   XTIT,    ]99. 
Collins,  Treacher,  Transactions  Ophthalmological  Society,  1903,  XXIII,  390. 
Hirschberg,  Centralb.  f.  p.  Angenh.,  January,  1904,  p.  12. 
Knapp,   Transactions   American   Ophthalmological   Society,   1870.    I,   121. 
Leber,  Unpublished  case  cited  by  Stargardt. 

Mayou,   Transactioris   Ophthalmological   Society,   1904,   XXIV,   142. 
Oatman,  Arch,  of  Oph.,  1907,  XXXVI,  554. 
Stargardt,   Archiv   f.   Ophthalmologic,   1909,   LXXI,   543. 
Stephenson,   Transactions   Ophthalmological   Society,   1904,   XXIV,   144. 
Stock,   Klin.   med.    f.   Angenh.,   XLVI,    1,   p.   225. 

Vogt.    Ueber    familiare    amarurotische    Idiote,    Monatasschr.    f.    Psvch.    u.    Xeurologie, 
1905,  Band  XVIII,  p.  161;  Band  XXII,  p.  495. 

Amaurotic  Family  Idiocy  (Tay's  Disease).  Warren  Tay,  in  1881, 
was  the  first  to  describe  a  peculiar  disease  of  the  cerebrospinal  system,  oc- 
curring exclusively  In  children  and  attended  by  Idiocy  and  blindness.  All 
indubitable  cases  so  far  reported  have  occurred  among  Hebrews.     The 


^Usually,  it  is  uncertain  that  microscopic  changes  found  in  the  rods  and  cones  are 
due  to  disease.  Post-mortem  degeneration  and  preparing  the  eye  for  examination  cause 
these  delicate  structures  to  disintegrate. 


DEGENERATIONS    OF    THE    RETINA.  201 

disease  tends  to  attack  several  members  of  a  childship  at  about  the  same 
period  of  life.  The  child  remains  healthy  until  from  four  to  six  months 
of  age  when  it  becomes  apathetic  and  sleeps  most  of  the  time.  Gradually, 
paresis  or  paralysis  of  the  general  muscular  system  develops  and  the  pa- 
tient is  unable  to  sit  up  or  to  support  the  head.  Certain  muscles  may  be 
spastic  instead  of  flaccid.  Abnormal  sensitiveness  to  touch  and  sound 
(hyperacusis)  often  appears  and  when  present  increases  the  difl^culty  of 
an  ophthalmoscopic  examination.  Occasionally,  convulsions  occur  but  are 
not  considered  a  symptom  of  the  disease.  Two  cases  have  been  reported 
in  which  hearing  was  lost  (Sachs).  Coincident  with  the  general  nervous 
affection,  eye  symptoms  develop  which  are  peculiar  to  amaurotic  family 
idiocy.  Both  eyes  are  symmetrically  affected.  Failure  of  vision  is  soon 
followed  by  blindness.  The  pupils  are  dilated  and  sluggish  and,  in  the 
last  stage  of  the  disease,  irresponsive  to  light.  Rolling  the  eyes,  or  nys- 
tagmus is  common.  The  ophthalmoscopic  picture  is  characteristic  of  the 
affection.  I'he  macula  is  occupied  by  a  broad,  whitish  disc  in  the  center 
of  which  the  fovea  appears  as  a  reddish  spot,  producing  the  same  appear- 
ance as  seen  in  closure  of  the  central  artery.  The  optic  disc  becomes  pale 
and  ultimately  white.  It  is  not  surrounded  by  retinal  opacity.  In  all  cases 
which  I  have  examined  the  retinal  vessels  appeared  normal.  All  bodily 
functions  are  torpid,  especially  manifested  by  pulmonary  and  gastro- 
intestinal disturbances.  With  advance  of  cerebral  degeneration  the  child 
passes  into  absolute  imbecility.  The  processes  of  metabolism  are  seriously 
impaired  and  a  condition  of  extreme  marasmus  develops  which  nearly 
always  terminates  in  death  within  two  years.  Sachs  has  met  but  one 
example  of  the  disease  which  extended  beyond  two  years.  In  this  case 
other  members  of  the  childship  were  affected.  When  seen  by  Sachs  the 
child  had  attained  the  age  of  5  5^  years  and  death  did  not  appear  to  be 
imminent. 

Etiology.  The  etiology  of  amaurotic  family  idiocy  is  unknown. 
The  most  remarkable  feature  of  the  disease,  as  pointed  out  by  Carter, 
is  racial  influence.  In  New  York,  which  has  a  Hebrew  population  of  a 
million,  I  see  from  one  to  three  cases  of  the  disease  yearly,  but  have  never 
met  a  suspicious  instance  among  gentiles.  This  racial  peculiarity  appears 
to  exclude  either  toxic,  bacterial  or  accidental  origin  for  the  disease.  None 
of  the  authentic  cases  has  been  attributed  to  syphilis.  Consanguinity  of 
parents  is  noted  in  some  cases  and  undoubtedly  exists  in  others  in  which  it 
is  denied.  It  is  uncertain  whether  the  disease  is  congenital  or  acquired, 
but  probably  the    former.      The   suggestion   that   mother's  milk  may   be 


202  THE    FUNDUS    OCULI. 

causative  is  disproved  by  several  of  Sack's  cases  who  were  fed  by  strange 
wet  nurses.  Sachs  considers  amaurotic  idiocy  as  some  congenital  defect 
which,  at  a  certain  age,  arrests  development.  This  is  a  modification  of 
Edinger's  Ersatztheorie,  according  to  which  tissue  endurance  is  too  feeble 
to  withstand  the  physiologic  activity  of  life.  A  family  taint  of  insanity  in 
both  parents  has  been  suggested  as  causative.  Injury  of  the  mother 
during  pregnancy  has  been  reported  in  several  instances  of  the  disease. 

NIoRBiD  Anatomy.  The  first  anatomiical  examination  was  made  by 
Sachs  in  1887.  There  was  no  evidence  of  inflammatory  action.  The 
brain  was  unduly  firm  with  the  consistence  of  gutta-percha.  The  essential 
histologic  feature  of  the  disease  was  found  in  the  cortex  and  consisted 
of  degenerative  changes  in  the  ganglion  cells.  Hirsch  was  the  first  to 
show  that  the  degeneration  of  ganglion  cells  found  in  the  brain  extended 
throughout  the  grey  matter  of  the  entire  central  nervous  system.  Ward 
Holden  in  1898,  was  the  first  to  demonstrate  that  the  blindness  and  peculiar 
appearance  in  the  retina  were  caused  bv  degeneration  of  the  ganglion 
cell  and  nerve  fiber  layer.  It  is  assumed  that  the  degeneration  is  primary  in 
the  cell  and  secondary  in  the  fiber  or  axon.  The  ganglion  cells  were  in- 
creased in  size  and  contained  a  globular  nucleus.  Cell  membrane  and 
cytoreticulum  were  intact,  but  the  Xissl  bodies  had  entirely  disappeared.* 

The  macular  opacity  seen  with  the  ophthalmoscope  has  been  attributed 
to  edema  of  the  retina,  but  it  is  improbable  that  any  real  edema  exists, 
the  opacity  being  caused  solely  by  the  dead  ganglion  cells  which  are  eight 
or  ten  layers  thick  in  this  region,  while  in  other  parts  of  the  retina  they 
consist  of  a  single  layer.  In  some  cases  a  fold  in  the  retina  involving  the 
macula  has  been  described  (Fig.  130),  but  this  is  a  post-mortem  artefact 
common  in  the  eyes  removed  many  hours  after  death.  Likewise,  the 
presence  of  a  hole  in  the  macula  was  supposed  to  explain  the  red  spot, 
but  this  also  is  an  artefact  produced  by  rough  manipulation  or  laboratory 
processes. 

I  microscopically  examined  one  case  of  this  disease  in  which  the  eye 
was  removed  eight  hours  after  death. t  The  macula  was  thrown  into  a 
sharp  post-mortem  fold  (Fig.  130).  At  the  macula  the  external  granular 
layer  was  normal;  the  external  reticular  layer  was  not  edematous;  internal 
granular  and  ganglion  cell  layer  were  very  thick.     Owing  to  the  time  that 


'■'The  ganglion  cells  of   the  retina  undergo  rapid  post-mortem   disintegration,   and   ex- 
aminations must  be  made  almost  immediately  after  death. 

tFrom  the  service  of  Dr.  A.  E.  Davis,  New  York  Post  Graduate  Hospital. 


DEGENERATIONS    OF    THE    RETINA.  203 

elapsed  between  death  and  fixation  of  the  eye  by  preserv^atives,  it  cannot 
be  asserted  that  all  the  finer  cell  changes  were  due  to  disease. 

The  optic  nerve  was  atrophic.  The  atrophy  and  thinning  of  the 
nerve  fiber  layer  being  especially  marked  on  the  temporal  side  of  the  disc 
(Fig.    131). 

Stereogram  57.  Amaurotic  Family  Idiocy.*  Right  eyeground 
of  male  infant,  aged  10  months;  firstborn;  Russian  Hebrew;  parents 
are  second  cousins;  the  child  is  apathetic  and  appears  to  be  blind;  pupils  are 
dilated  and  very  sluggish;  a  paretic  condition  of  the  muscular  system  is 
shown  by  inability  of  the  child  to  sit  up  or  to  support  the  head,  which 
droops.  Ophthalmos-copic  Picture.  The  fovea  appears  as  a  maroon  col- 
ored spot,  surrounded  by  a  wide  zone  of  greyish-white  retina.  The  optic 
nerve  is  pallid,  especially  on  the  temporal  side.  The  retinal  vessels  appear 
normal  and  a  number  of  small  vessels  around  the  macula,  ordinarily  in- 
visible, are  brought  into  view  by  contrast  with  the  white  retina. 

Diagnosis.  Amaurotic  family  idiocy  is  diagnosticated  by  the  presence 
of  characteristic  fundus  changes  in  a  paralytic  infant,  and  by  the  history. 
It  is  said  that  the  cerebrospinal  degeneration  may  precede  the  eye  symptoms 
which,  however,  develop  later.  The  ophthalmoscopic  picture  differs  from 
that  of  central  artery  closure  in  that  the  vessels  are  not  narrowed,  or  the 
retina  opaque  around  the  nerve. 

Bibliography. 

Tay,  T.  Oph.  Soc,  vol.  I.  1881,  55. 

Sachs,  Jour.  ]Ment.  &  Nerv.  Dis.  1887.  541:    1892,  1903.    X.  V.  Med.  Jonr.  1896.  Deutsch. 

Med.  Woch.  1898;  1903.     Posey  and  Spiller.  "The  Eve  and  Nervous  Dis"  n    532 
Carter,  A.  of  Oph.  XXTTT.  1894. 
Edinger,    Parsons,   Path,   of   the   Eye,   IV,   1368. 
Hirsch,   Jonr.  of  AFent.  and  Nerv.   Dis..  1899.  538. 
Hirsch  and   Holden.   Jour.   Ment.  and  Nerv.   Dis.   1898,  XXV. 
Holden,  Trans.   .Am.   Oph.   Soc,  1898,  405 
Davis  and  Oatman,  Contrib.  to  Med.  &  Surg.  25th  Anniver.   N.  Y.   Post  Grad.   Med. 

School  and  Hospital,  1908. 


From  the  clinic  of  Dr.  P.  C.  Jameson;  Brooklyn  Eye  and  Ear  Hospital. 


Chapter  X. 
MISCELLANEOUS  AFFECTIONS  OF  THE  RETINA. 

Cyanosis  Retin.^.  Marked  distension  and  tortuosity  of  the  retinal 
vessels  unattended  by  hemorrhage,  exudation  or  edema,  occurs  in 
diseases  which  produce  general  venous  stasis.  1  his  retinal  condition  is  most 
frequently  seen  in  connection  with  the  general  cyanosis  incident  to  an 
open  foramen  ovale  in  which  there  is  admixture  of  venous  with  arterial 
blood  within  the  heart,  and  also  in  cases  of  pulmonary  stenosis.  Congenital 
cyanosis  has  been  classed  as  a  disease  under  the  name  of  Morbus  Ccvriileits, 
or  blue  disease,  and  the  subjects  of  the  affection  are  known  as  blue  children. 
The  congestion  seen  in  the  retina  exists  in  other  organs;  thus,  enlargement 
of  the  liver  and  spleen,  atelectasis,  pleural  effusion  and  edema  of  the  brain 
are  frequent.  The  disease  is  recognized  by  the  peculiar  livid  hue  of  the 
skin,  which  varies  from  a  dusky  tinge  to  a  deep  purple  color.  Although 
the  entire  surface  of  the  body  is  affected,  the  discoloration  is  most  marked 
on  the  lips,  cheeks  and  eyelids.  The  skin  is  dry  and  the  extremities  cold. 
Palpitation  and  dyspnea  are  always  present  and  are  greatly  increased 
by  excitement  or  exercise.  Somnolence  is  a  common  symptom.  Polycy- 
themia develops  in  cases  of  pulmonic  obstruction.  Increase  in  the  number 
of  red  blood  corpuscles  is  proportionate  to  the  cyanosis,  the  average  number 
present  being  about  7,000,000  (Tyson).  A  corresponding  increase  occurs 
in  the  amount  of  hemoglobin.    The  leucocytes  are  not  increased  in  numbers. 

The  duration  of  lite  varies.  One  half  of  all  cases  die  in  the  first 
year.  Other  cases  survive  until  adult  life.  Litten  mentions  a  case  which 
lived  to  the  age  of  25  years.  There  is,  of  course,  no  chance  of  recovery. 
The  most  dangerous  periods  of  life  for  cyanotic  cases  are  the  first  respira- 
tory act,  the  period  of  weaning,  first  dentition  and  during  any  intercurrent 
disease.  In  the  later  period  of  the  disease  the  retinal  vessels  may  become 
diseased  and  hemorrhages  occur.  Posey  reports  a  case  presenting  the  pic- 
ture of  a  neuroretinitis. 

Congestion  of  the  retinal  vessels  producing  the  ophthalmoscopic  pic- 
ture of  "  cyanosis  retinae  "  has  been  observed  in  emphysema  by  Stephen 
Mackenzie.     In  one  of  Hirschberg's  cases  of  general  and  retinal  cyanosis, 

204 


MISCELLANEOUS    AFFECTIONS    OF    THE    RETINA.  205 

there  were  pulmonary  emphysema  and  hypertrophy  of  the  heart  which  were 
due  to  kyphoskoliosis.  The  condition  of  intense  retinal  hyperemia  has 
been  seen  during  the  height  of  an  epileptic  seizure  and  also  in  certain 
intoxications   (Stergm.  60). 

Stereogram  58.  Retina  in  Congenital  Cyanosis  (Cyanosis 
Retinae).  Right  eyeground  of  a  "  blue  boy,"  eleven  years  of  age.  Since 
birth  the  skin  has  been  cyanotic.  The  blue  color  is  especially  marked  on 
the  lips  and  eyelids.  Owing  to  dyspnea  he  has  never  been  able  to  play 
like  other  children.  A  physical  examination  indicated  patent  foramen  ovale 
and  pulmonic  stenosis.      Refraction  is  emmetropic.     Vision  20/20. 

Ophthalmoscopic  Examination.  The  entire  fundus  has  a  deep 
red  color.  The  optic  disc  appears  reddened  except  in  the  physiologic 
excavation.  Both  veins  and  arteries  are  very  tortuous  and  somewhat 
dilated.  The  arteries  are  not  sclerosed,  as  shown  where  they  cross  over 
a  vein  without  indenting  the  latter. 

Diagnosis.  The  diagnosis  of  cyanosis  retina^  rests  upon  the  presence 
of  general  cvanosis.  The  appearance  of  the  fundus  is  not  very  character- 
istic of  the  disease.  For  example,  were  this  eye  hyperopic  it  could  not  be 
ophthalmoscopically  diagnosticated  from  the  picture  sometimes  presented 
by  that  condition.  Arteriosclerosis  may  be  excluded  by  the  perfectly  nor- 
mal appearance  of  the  vessels.  Cyanosis  is  differentiated  from  inflam- 
matory conditions  by  normal  vision  and  absence  of  hemorrhage,  exudation 
and  retinal  edema. 

Bibliography. 

Von  Ammon.  Nor.  &  Oliv.,  vol.  Ill,  p.  433. 
Stephen    Mackenzie,   Trans.   Oph.   Soc,   1882-3. 
Posev.  Trans.  Am.  Oph.  Soc,  X,  p.  632. 
Hirschberg,  Arch.   f.   Oph.   LIX.  p.   ]3L 
Litten.   Deutsch.    I\Ied.   Wochenshrf..   1887.  p.   144. 
Tyson,   Trans.   Am.   Oph.   Soc.   1908. 

Tral^matic  Edema  of  the  Retina  (Commotio  Retinae).  Tempo- 
rary blindness  varying  in  duration  from  a  few  moments  to  several  hours 
nearlv  always  follows  violent  concussion  of  the  eye.  This  condition  of 
traumatic  amblyopia  may  appear  and  disappear  without  visible  alteration 
in  the  retina.  In  other  cases  deterioration  of  vision  persists  and  the 
retina  undergoes  characteristic  changes  consisting  of  an  opacity  which 
extends  more  or  less  completely  over  the  posterior  polar  zone.  In  well 
marked  cases  the  appearance  of  the  retina  resembles  somewhat  the  con- 
dition which  follows  closure  of  the  central  artery.     The  opacity,  which  is 


206  THE    FUNDUS    OCULI. 

due  to  edema  of  the  retina,  is  most  conspicuous  where  the  membrane  is 
thickest,  namely,  around  the  fovea  centralis  and  optic  disc.  The  fovea 
itself,  however,  is  the  thinnest  portion  of  the  retina  and  may  permit  the 
choroid  to  shine  through  and  produce  a  "  cherry-red  "  spot.  In  other 
cases  the  edematous  retina  may  overlie  the  fovea  so  that  it  is  nearly  or 
completely  obscured.  Sometimes  the  retina  at  the  fovea  is  torn  and  an 
actual  hole  exists.  This  accident  usually  occurs  in  myopic  eyes.  In  ail  cases 
of  commotio  retinae  which  I  have  observed  the  retinal  opacity  at  the  macula 
was  more  sharply  outlined  than  in  closure  of  the  central  artery.  The  blood 
vessels  usually  are  dilated  and  the  perivascular  lymph  channels  may  be 
distended.  The  visible  retinal  changes  vary  from  an  almost  imperceptible 
cloudiness  at  the  macula  to  dense  opacity  and,  in  rare  cases,  hemorrhages. 

The  term  "  commotio  retinae  "  formerly  was  applied  to  any  obscure 
case  of  visual  impairment  following  injury  to  the  head.  Further  investiga- 
tion established  edema  of  the  retina,  especially  of  the  nerve  fiber  layer,  as 
the  chief  anatomic  alteration,  although  the  mechanism  by  which  the  edema 
is  produced  is  not  fully  determined.  Berlin,  who  first  separated  these 
cases,  thought  the  retinal  edema  was  caused  by  suprachoroidal  hemorrhage, 
a  view  soon  abandoned.  The  majority  of  writers  now  agree  that  the 
first  result  that  follows  a  blow  to  the  eye  is  retinal  ischemia  due  to 
spasm  of  the  blood  vessels.  The  angiospasm  is  immediately  followed  by 
vascular  dilation  and  transudation  of  serum.  Denig,  as  the  result  of 
animal  experimentation,  states  that  a  violent  blow  drives  the  vitreous 
against  the  retina,  rupturing  the  internal  limiting  membrane  and  forcing  the 
vitreous  fluid  between  the  nerve  fibers  and  along  the  fibers  of  Miiller. 
Traumatic  retinal  edema  may  occur  with  rupture  of  the  choroid 
or  other  effects  of  blunt  force.  The  most  commonly  associated  con- 
ditions are  opacity  of  the  cornea,  miosis,  mydriasis,  lacerations  of  the  iris, 
dislocation  or  opacltv  of  the  lens  and  hemorrhage. 

In  the  following  case  the  macular  changes  found  on  microscopic  ex- 
amination were  regarded  as  those  of  traumatic  edema  of  the  retina. 

A  man  received  a  nonperforating  blow  in  the  eye.  Twenty-four 
hours  later  the  eye  was  glaucomatous  with  widely  dilated  pupil.  The  lens 
was  dislocated  forwards.  Vision  reduced  to  movements  of  the  hand. 
Details  in  the  fundus  were  indistinguishable  although  the  reflex  from  the 
macular  region  was  much  lighter  in  color  than  from  other  regions.  Iri- 
dectomy and  extraction  failed  to  relieve  the  condition  and  the  eye  was 
enucleated  fourteen  days  after  the  injury.  Microscopic  examination.  The 
iris   is  thrown   into  folds  and  lies  in  the   angle  of  filtration   where   it   is 


Fig.  130 — Amaurotic  Family  Idiocy. 
Macular  Opacity  Due  to  Death  of  the 
Thick  Layer  of  Ganglionic  Nerve  Cells. 
The  Retinal  Fold  is  an  Artefact. 


Fig.  131 — Amaurotic  Family  Idiocy. 
Optic  Atrophy,  which  is  Most  Marked 
on  the  temporal  Side  of  the  Disc,  the 
Left  Side  in  the  figure. 


Fig.  131. 


Fig.  132— Glacoma  Following 
Injury.  The  Iris,  Thrown  into  Folds 
and  in  Contact  with  the  Cornea,  Oc- 
cludes  the   Filtration   Angle. 


Fig.  132. 


MISCELLANEOUS    AFFECTIONS    OF    THE    RETINA.  207 

closely  pressed  against  the  cornea  (Fig.  132).  The  condition  of  the 
macula  is  shown  in  Fig.  20.  The  retina  is  edematous,  the  edema  being 
limited  to  the  nerve  fiber  and  internuclear  layers.  The  fovea  is  widened 
and  contains  a  serous  effusion. 

In  commotio  retins  it  is  probable  that  minute  changes  occur  in  the 
nervous  tissue  not  demonstrable  with  the  microscope.  Central  vision  is 
always  impaired  and  the  visual  fields  are  somewhat  concentrically  con- 
tracted. 

Tn  cases  where  the  traumatism  results  only  in  edema  the  prognosis 
is  good  and  vision  is  fully  restored.  Within  a  week  or  two  the  retinal 
opacity  disappears,  at  which  time  white  spots  situated  in  the  deeper 
layers  of  the  retina  come  into  view.  In  a  short  time  these  also  are  re- 
mov^ed  and  no  trace  of  the  effusion  remains.  Exceptionally,  slight  pig- 
mentation results,  in  which  case  it  is  supposed  that  the  choroid  participates 
in  the  disturbance.  When  concussion  results  in  actual  rupture  of  the 
retina  and  a  hole  forms  at  the  macula,  the  prognosis  is  less  favorable. 
The  hole  does  not  always  result  directly  from  concussion,  but  may  develop 
later  from  excessive  edema.  Holden  reports  a  case  in  which  three  weeks 
after  a  blow  the  retina  was  intact,  but  two  months  later  a  typical  hole  de- 
veloped at  the  macula. 

Stereogram  59.  Tralmatic  Edema  of  the  Retina  (Commotio 
Retinae).  Right  fundus  oculi  of  a  barkeeper,  33  years  of  age.  Seven  days 
ago  he  received  a  violent  fist-blow  on  the  right  temple,  immediately  fol- 
lowed by  loss  of  vision  in  the  right  eye.  At  the  present  time,  with  this  eye 
he  can  count  fingers  at  five  feet.  Retinal  edema  is  marked  around  the 
nerve  and  macula  and  slightly  developed  throughout  the  rest  of  the  pos- 
terior zone.  The  macula  is  occupied  by  a  sharply  defined,  rounded, 
grev  disc  in  the  center  of  which  the  fovea  appears  as  a  dark  red  spot. 
Around  the  nerve  the  edema  is  greatest  on  the  nasal  side  where  it  nearly 
hides  the  outline  of  the  disc.  The  veins  are  full  and  darkened.  Arteries 
and  veins  on  and  near  the  disc  are  bordered  by  delicate  white  lines,  sup- 
posed to  represent  an  effusion  into  the  perivascular  sheaths.  The  nerve 
anci  vessels  present  the  appearance  of  commencing  neuritis. 

Improvement  in  vision  immediately  ensued.  The  edema  rapidly 
disappeared,  the  macular  spot  grew  indistinct  and  within  a  week  the  fundus 
exhibited  little  evidence  of  injury. 

Diagnosis.  In  the  great  majority  of  cases  a  history  of  injury  at 
once  leads  to  the  correct  diagnosis.  Occasionally,  however,  the  true  history 
is  suppressed.     In  both  traumatic  edema  and  closure  of  the  central  artery 


208  THE    FUNDUS    OCULI. 

there  may  be  retinal  opacity  and  a  red  spot  at  the  macula.  In  arterial 
closure  the  arteries  are  greatly  narrowed  or  invisible  while  in  traumatic 
edema  the  vessels  are  overfull.  In  traumatic  edema  the  macular  opacity 
is  apt  to  be  more  sharply  defined  than  in  closure.  Amaurotic  idiots  pre- 
sent macular  opacity  and  a  red  fovea,  but  the  subjects  of  this  disease  always 
are  infants  affected  by  a  general  paresis. 

Bibliography. 

Berlin,  Kl.  F.  f.  Augk.,  XI,  1873 :    Graefs  Saemisch.  VI,  1880. 

Ward  Holden,  Trans.  Oph.  Sec.  N.  Y.  Acad.  Arch,  of  Oph.  XXXIII,  1904,  p.  190. 

Denig,  Arch,  of  Oph.  XXVI,  1897,  p.  377. 

Toxic  Amaurosis  and  Amblyopia. 

Stereogram  60.  Retinitis  in  Wood  Alcohol  Poisoning.  (Con- 
gestive Edema  of  the  Retina).*  Left  eyeground  of  a  young  man  blind 
from  acute,  methyl  alcohol  poisoning.  After  working  three  weeks  shel- 
lacing the  interior  of  beer  vats,  where  he  inhaled  the  concentrated  fumes 
of  wood  alcohol  in  which  the  shellac  was  dissolved,  he  was  seized  with 
violent  vomiting  and  24  hours  later  became  totally  blind.  The  pupils 
were  widely  dilated  and  unresponsive  to  light.  About  one  month  later 
he  could  count  fingers  with  the  left  eye  but  perception  of  light  never  re- 
turned in  the  right.  Both  optic  nerves  gradually  atrophied  and  permanent 
blindness  ensued. 

The  ophthalmoscopic  picture,  taken  in  the  early  stage,  indicates  an 
inflammatory  edema  of  the  retina.  Both  arteries  and  veins  are  dilated 
and  tortuous.  Extensive  effusion  of  serum  into  the  retina  has  occurred 
along  the  larger  vessels.  There  is  no  optic  neuritis,  although  edema  from 
the  retina  has  extended  o\'er  the  upper  and  lower  margins  of  the  disc. 
Were  this  nerve  swollen,  the  arteries  would  be  compressed  and  narrowed. 

Diagnosis.  The  ophthalmoscopic  picture  is  not  characteristic  and 
the  diagnosis  in  wood  alcohol  poisoning  is  made  chiefly  from  the  history. 
In  acute  wood  alcohol  amaurosis,  blindness  develops  rapidly  in  both  eyes, 
hours  or  days  after  a  debauch.  Pupils  dilated  and  fixed.  The  fundus  pic- 
ture varies,  there  may  be,  (1)  normal  nerve  and  retina;  (2)  congested 
nerve  and  retina;  (3)  retinal  ischemia.  Usually  there  is  temporary  im- 
provement followed  by  blindness  or  constricted  fields.  In  uremic  amaurosis 
the  pupils  usually  respond  freely  to  light  while  in  wood  alcohol  poisoning 
they  are  immobile.      If  uremic  coma  coexists  with   amaurosis,   casts  and 

*  From  the  service  of  Dr.  David  Webster,  Manhattan  Eye,  Ear  and  Throat  Hospital. 


L.E. 


P.E. 


, dp  d  ^  ^jol^K^^-Tp  if)  ■vL^.  A*  >t'>  .y?  i'(>  J§  :/«  "^^^j/-'^^^ 


AS  e-^Cf-wlvic    ClrviXrcc  , 


Fig.  133  (a). 


Fig.  133  (b). 


MISCELLANEOUS    AFFECTIUXS    OF     J  111-.    Ki:ilNA.  209 

albumin  may  temporarily  disappear  from  the  urine.  Retinitis  may  or  may 
not  coexist.  Amaurosis  from  cerebral  or  spinal  disease  develops  gradually 
and  is  steadily  progressive. 

^cute  poisoning  by  wood  alcohol  is  not  rare.  Usually,  it  follows  a 
debauch  upon  some  cheap  liquor  composed  largely  of  methyl  alcohol,  or 
from  prolonged  inhalation  of  its  fumes.  In  the  lesser  degrees  of  poisoning 
central  scotoma  may  be  demonstrated.  In  late  cases  concentric  contraction 
of  the  white  field's  may  exist.  Blindness  may  not  develop  for  several  days 
after  ingestion  of  the  poison.  Paralysis  of  the  face  or  limbs  and  loss  of 
intelligence  are  not  uncommon  in  wood  alcohol  poisoning.  Temporary 
improvement  of  vision  often  ensues,  followed  by  totil  blindness.  Other 
cases  recover  with  more  f)r  less  contraction  of  the  fiekh  (Fig.  133a). 

Tyson*  has  called  attention  to  chronic  toxic  amblyopia  which  is  not 
uncommon  in  those  who  work  with  colors  or  varnishes  dissolved  in  wood 
alcohol. 

As  demonstrated  bv  Holden,  the  blindness  produced  bv  methyl  alco- 
hol is  due  to  destruction  of  the  ganglion  cells  i:i  the  retina.  In  addition 
to  its  action  on  nerve  tissues,  it  exerts  a  powerful  influence  on  the  retinal 
blood  vessels  manifested  however,  by  widely  divergent  ophthalmoscopic 
'pictures.  In  one  type  a  condition  of  retinal  anemia  with  narrowed  vessels 
exists,  in  another  the  vessels  are  dilated  and  the  retina  acutelv  congested. 
Again,  there  mav  be  absolute  blindness  v,-'thout  visible  fundus  changes. 

Chronic^  Alcoholism.  Amblyopia  due  solely  to  chronic  alcoholism 
is  extremely  rare.  It  differs  in  no  respect  from  tobacco  amblvopia,  ex- 
cept that  other  symptoms  of  alcoholism  mav  coexist. 

Tobacco  Amblyopia.  The  intemperate  use  of  tobacco  and  alcohol 
usually  go  hand  in  hand  but,  clinically,  this  form  of  amblyopia  occurs  almost 
exclusively  among  those  who  for  vears  have  been  excessive  consumers  of 
tobacco.  The  symptoms  de\'elop  slowlv.  Both  eves  are  uniformly  af- 
fected, l^he  first  complaint  is  of  foggy  vision  and  of  "  dazzling  "  when 
in- a  strong  light.  Day  blindness  (nyctalopia)  is  a  prominent  symptom 
and  its  presence  always  suggests  tobacco  poisoning.  Gradually,  central 
vision  deteriorates  and  after  a  time  reading  becomes  impossible  although 
the  peripheral  fields  of  vision  are  retained.  The  visral  fields  present  a 
central  color  scotoma  for  red  and  green  with  a  relative  central  scotoma 
for  white  (Fig.  133b).  The  scotoma  in  tobacco  amblyopia  corresponds  to 
the  distribution  of  the  maculopapillary  bundle  of  nerve  fibers,  /.  e.,  a 
horizontally    oval    area    which    just    embraces    the    macula     and    optic 

*Tyson,  Arch,  of  Ophthalmology,  Sept.  1912. 
14 


210  THE    FUNDUS    OCULI. 

nerve.  If  the  patient  persists  in  the  use  of  tobacco  the  relative  white 
scotoma  becomes  absolute  and  central  vision  is  lost.  The  peripheral 
fields,  however,  are  not  affected  and  complete  blindness  does  not  occur.  If 
the  use  of  tobacco  is  discontinued  when  toxic  symptoms  first  develop,  re- 
covery may  be  expected. 

The  pathologic  lesion  is  generally  regarded  as  a  primary  inflam- 
mation of  the  maculopapillary  bundle  of  nerve  fibers  in  the  optic  nerve. 
This  cannot  be  considered  as  fully  established,  particularly  as  blindness 
in  methyl  alcohol  poisoning  is  supposed  to  be  caused  by  destruction  of 
the   ganglion   cells   in   the   retina. 

loDiFORM  Poisoning.  Poisoning  by  iodiform,  which  at  one  time  was 
common,  produces  eye  symptoms  almost  identical  with  those  of  alcohol 
and  tobacco. 

Quinine  Amblyopia.  Quinine  in  poisonous  doses  produces  extreme 
ischemia  of  the  retina  and  blindness.  As  a  rule,  vision  returns,  although 
if  poisoning  has  been  severe  permanent,  concentric  constriction  of  the 
visual  fields  results.  In  experimental  poisoning  with  quinine,  Holden 
found  narrowing  of  the  retinal  vessels  and  degeneration  of  the  ganglion 
cells.  It  is  not  clear  whether  the  ganglion  cell  changes  were  due  to 
direct  action  of  quinine  or  to  malnutrition  from  anemia  of  the  retina.  Par- 
sons concluded  from  experiments  on  animals  that  the  retinal  ischemia  in 
quinine  poisoning  was  due  to  withdrawal  of  blood  from  the  surface  and 
its  accumulation  in  the  great  abdominal  veins. 

Salicylic  Acid  may  produce  temporary  amblyopia  resembling  quinine 
poisoning  as  described  above.  Other  drugs  which  may  produce  amblyopia 
with  a  pale  disc  and  retina!  anemia  are  felix  mas,  oil  of  wintergreen,  co- 
caine, ergot,  antifebrine,  acetanalid,  adrenaline,  nitorbenzol  and  chlorate 
-of  potash. 

Amblyopia,  usually  accompanied  by  congestion  of  the  nerve  and 
retina,  occurs  in  poisoning  by  phosphorus,  scamonium,  pomegranate,  col- 
ocynth,  ptomaines  and  aniline  oil.  The  last  named  may  exhibit  a  violet 
fundus  and  black  vessels. 

Amblyopia  has  been  reported  from  overdoses  of  sulphuretted  hydro- 
gen, carbolic  acid  and  other  drugs.  The  intramuscular  injection  of  arsenic 
and  anilin  preparations  for  cure  of  syphilis  has  been  followed  by  optic 
atrophy. 

The  fundus  picture  of  lead  amblyopia  is  inconstant  and  varies  from 
choked  disc  with  retinal  hemorrhage  to  blindness  without  ophthalmo- 
scopically  visible  changes. 


Fig.  134 — Retinitis  Prolifcrans.  Dense 
Masses  of  Connective  Tissue  in  the 
Inner   Retinal   Layers. 


FiR.  i;j4. 


Fig.  135  —  Retinitis  Proliferans. 
Thin  Membrane  of  Connective  Tissue 
arising  from  the  Retina  and  Extend- 
ing Forward  into  the  Vitreous. 


Fig.  135. 


Fig.  136 — Retinitis  Prolifer- 
ans. Connective  Tissue  Forma- 
tion on  Disc.  Thick  Mem- 
branes Originating  therefrom 
are  Continued  Forward  into  the 
Vitreous. 


Fig.  136. 


.Misci:i.L.\Ni:ors  affections  of  thk  retina.  211 

Colored  vision  {chromolops'ui)  may  be  caused  by  drugs.  Santonin 
usually  produces  yellow  vision,  less  frequently,  red  or  green  vision.  Chro- 
motopsia  mav  be  caused  also  by  chromic  acid,  picric  acid,  amyl  nitrite, 
cannabis  indica,  digitalis,  carbonic  acid,  tobacco  and  coffee.  Temporary 
scotoma  may  be  due  also  to  coffee. 

Retinitis  Proliferans.  In  1869  Jager  illustrated,  and  in  1876 
Manz  named,  the  condition  known  as  retinitis  proliferans.  The  essential 
feature  of  this  disease  is  the  presence  of  dense  masses  of  connective  tissue 
(F^ig.  1.34)  or  of  thin  membranes,  which  originate  in  the  retina  and  extend 
torward  into  the  vitreous  (Pig.  135).  These  sheets  of  connective  tissue 
usually  arise  from  the  disc  or  its  vicinity  and  are  attached  anteriorlv  to  the 
ora  serrata.  Nearly  all  authors  regard  hemorrhage  as  an  essential  pre- 
liminary in  the  formation  of  retinal  membranes,  which  are  supposed  to  be 
metamorphosed  fibrinous  coagula.  As  stated  by  Flemming,  however,  some 
additional  factor  is  required  to  convert  blood  clots  into  membranes.  Pro- 
liferation of  neuroglia,  especially  of  Miiller's  fibers,  has  been  accepted  by 
some  as  the  source  of  the  new  tissue,  but  extensive  proliferation  of  neuroglia 
is  an  improbable  event.  A  more  reasonable  origin  for  the  membranes  is 
found  in  proliferation  of  the  connective  tissue  (adventitia)  which  surrounds 
the  vessels  on  the  disc  (Wehrli,  Romer,  Parsons).  Under  proper  stimu- 
lation this  tissue,  like  connective  tissue  elsewhere,  actively  proliferates. 
In  a  similar  manner,  connective  tissue  around  a  retinal  vessel  may  produce 
enormous  hypertrophy  of  the  inner  layer  of  the   retina    (Fig.    134). 

It  is  not  unusual  to  find  tibrous  membranes  in  eyes  which  have  been 
enucleated  for  injury  attended  by  intraocular  hemorrhage,  particularly 
when  a  foreign  body  is  retained  within  the  eye.  Figure  136  represents  a 
section  from  an  eye  in  which  a  piece  of  stone  was  imbedded  for  eight  months 
in  the  posterior  extremity  of  the  ciliary  body.  There  Is  enormous  prolifera- 
tion of  connective  tissue  on  the  disc.  The  same  tissue  is  continued  into  the 
vitreous  in  the  form  of  thick  membranes.  In  this  case  the  foreign  body 
which  stimulated  proliferation  was  remote  from  the  nerve. 

Flemming's  idea  that  the  hyaloid  membranes  are  concerned  in  retinitis 
proliferans  appears  true  for  certain  cases  In  which  the  vitreous  septa  ap- 
pear to  form  a  foundation  on  which  extraneous  matter  Is  deposited 
(Fig.  137). 

Figure  137  Is  from  a  case  of  typical  traumatic  retinitis  proliferans 
preceded  by  hemorrhage.*  A  piece  of  steel  entered  through  the  cornea  and 
lodged  In  the  retina  near  the  disc.     The  Intraocular  hemorrhage  which 

*From  a  case  which  I  microscopically  examined  for  Dr.  Dunbar  Roy,  of  Augusta,  Ga. 


212  THE    FUNDUS    OCULI. 

resulted  prevented  an  ophthalmoscopic  examination.  The  steel  was  re- 
moved by  magnet  and  the  wound  healed.  Six  months  later  the  eye  was 
enucleated  for  a  burn  of  the  cornea.  The  microscopic  examination  showed 
a  funnel-shaped  fibrous  membrane,  the  apex  of  which  was  attached  to  the 
retinal  wound,  near  the  nerve,  and  the  expanded  edge  to  the  ora  serrata 
(Fig.  1.38).  It  is  interesting  to  note  that  a  band  of  connective  tissue  ex- 
tended between  the  disc  and  posterior  attachment  of  the  membrane. 

Retinitis  proliferans  is  uncommon.  Weeks,  among  24,000  patients 
met  but  two  examples.  The  condition  may  be  due  to  traumatism  or  to  some 
constitutional  dyscrasia.  Weeks  speaks  of  syphilis  as  a  common  cause. 
Spontaneous  cases  occur  in  ill-nourished  young  people,  usually  males. 
Frequently,  both  eyes  are  aflected.  If  spontaneous  cases  are  preceded  by 
hemorrhage  some  form  of  vascular  degeneration  may  be  assumed.  Ex- 
ceptionally, contraction  of  the  membranes  leads  to  detachment  of  the 
retina    (Scott). 

The  prognosis  is  unfavorable.  Usually  the  eye  becomes  soft  and 
phthisical  although,  as  in  other  forms  of  intraocular  hemorrhage,  glaucoma 
may  develop. 

Stereogram  61.  Retinitis  Proeiferans.  Right  eyeground  of  a 
man  aged  28  years.  He  is  a  poorly  nourished  specimen  of  a  city  de- 
generate; blood  pressure  178  mm.  Hg. ;  surface  arteries  palpable;  urine 
contains  a  trace  of  albumin.  Has  often  been  severely  clubbed  by  the  po- 
lice and  beaten  in  fistic  encounters.  Following  such  an  encounter  two 
years  ago,  vision  became  poor  in  the  right  eye.  Ophthalmoscopic  Examina- 
tion. The  left  eye  contains  no  membranes  but  a  small  hemorrhage  has 
occurred  between  the  macula  and  disc.  The  right  eye  contains  a  number  of 
thin  membranous  formations,  attached  to  the  disc,  some  of  which  extend 
forward  beyond  the  limits  ot  the  ophthalmoscopic  field.  The  upper  of 
these  new  formations  appears  to  be  a  solid  mass  of  connective  tissue  covered 
with  adventitious  blood  vessels.  The  membranes  are  greyish-white  in 
color,  very  dense  at  the  disc  but  thin  and  semitransparent  anteriorly.  They 
are  immovable  and  do  not  float  about  in  the  vitreous  v/hen  the  eye  is  moved. 

Diagnosis.  The  ophthalmoscopic  picture  of  retinitis  proliferans  is 
unmistakable.  These  membranes  are  distinguished  from  detachment  of 
the  retina  by  their  whitish  color,  membranous  form  and  attachment  to  the 
nerve.  Occasionally,  retinitis  proliferans  leads  to  detachment.  These 
formations  differ  from  vitreous  opacities  of  inflammatory  origin  by  their 
color  and  immobility. 


Fig.   137 — Retinitis   Proliferans.     The   Fibrous  Septa  of  the  Vitreous  appear  to  have 
formed  a  Foundation  on  which  Extraneous  Matter  has  been  Deposited. 


Fig.  1G8. 

Fig.  1?,S — Retinitis  Proliferans.  From  same  specimen  as  Fig.  137.  The  Mem- 
branous Formation,  arising  from  near  the  Nerve  Head,  expands  Funnel-shaped  to  the 
Ora  Serrata  and  might  be  mistaken  for  a  Visible  Hyaloid  Canal. 


MISCELLANEOUS    AFFECTIONS    OF     llli.    RETINA.  213 

Bibliograpliy. 

Jager,  Handallas.  1869. 

Manz.    A.   f.   Oph..   XX 11.  ?,.   is76:    XXVT,  2.   isso 

Flemming    T    O.   Soc,   X\T1I,   ISDS. 

Wchrli.   A.   f.    Aughn..   XXXVI F.   189S 

Eomer.  A.   f.  Oph..  LIJ.  3,  190L 

Parsons,   Path,  of   the  Eye,  vol.  2,  p.  614. 

Weeks.  Text   Book.   1910.  p.  458;    Trans.  Am.  Oph.   Soc.  1897. 

Scott.  T.  O.  Soc.  XXTIT,  1903.  p.  71. 

Retinitis  Striata  and  Angioid  Streaks  in  the  Retina.  All 
forms  of  striate  conditions  in  the  fundus  have  been  described  as  retinitis 
striata  but  this  name  should  be  reserved  for  two  fairly  well  defined  clinical 
pictures,  now  designated  respectively  as  retinitis  striata  and  angioid  streaks. 

Retinitis  striata  is  the  term  applied  by  Xagle,  in  1864,  to  a  peculiar 
affection  apparently  allied  to  retinitis  proliferans.  It  is  characterized  by 
the  presence  of  branching  stride  in  the  retina  situated  behind  the  retinal 
vessels  and  in  front  oi  the  tessellated  epithelium.  The  streaks  vary  in 
width  from  one  to  three  times  the  diameter  of  a  primary  retinal  vessel, 
are  bluish-  or  yellowish-white  in  color  and  not  infrequently  bordered  or 
covered  by  a  dark  brown  pigment.  The  course  pursued  by  the  striae  is 
inconstant;  they  may  radiate  from  the  disc  in  the  most  eccentric  directions 
(Dunn)  or  they  may  traverse  the  fundus  diagonally  in  long,  straight 
lines,  the  general  direction,  however,  being  from  periphery  to  center. 
The  vessels  mav  appear  normal  or  sclerosed;  in  some  cases  they  have  been 
reported  as  extremely  tortuous  (Gloor;  Schilling).  In  most  reports  the 
vitreous  is  described  as  clear,  occasionally  as  turbid.  The  disease  does 
not  appear  to  have  any  constant  subjective  symptoms  such  as  disturbance 
of  the  color  sense,  hemeralopia,  nyctalopia,  etc.  Usually,  vision  is  con- 
siderably lowered  and  the  visual  field  irregularly  contracted,  but  these  symp- 
toms appear  to  bear  a  relation  to  the  amount  of  mechanical  destruction  of 
tissue.  The  retina  may  or  may  not  exhibit  opacity.  This  variety  of 
striate  retinitis  usually  is  confined  to  one  eye  and  in  many  cases  a  traumatic 
origin  can  be  traced. 

Angioid  streaks  in  the  retina  is  a  condition  closely  related  to,  if  not 
identical  with,  the  retinitis  striata  of  Nagle.  The  first  case  was  reported 
by  Plange,  to  which  Herman  Knapp  immediately  added  another  and  named 
the  disease  "  angioid  streaks  in  the  retina."  In  this  type  of  the  affection 
the  streaks  occupy  the  same  position  beneath  the  retinal  vessels  and  pursue 
a  similar  course  as  in  retinitis  striata.  They  differ,  however,  in  that  they  are 
narrower  and  consist  of  brownish  pigment  instead  of  white  tissue.     An  ex- 


214  THE    FUNDUS    OCULI. 

ception  as  regards  the  color  of  the  streaks  existed  in  a  case  by  Stephenson 
in  which  the  brown  stripes  were  bordered  on  one  side  by  a  broader  white 
stripe,  suggesting  a  transformation  of  angioid  streaks  into  white  striae 
(retinitis  striata).  Angioid  streaks  are  more  frequently  bilateral  than 
retinitis  striata.  Both  types  belong  to  early  adult  life,  while  some  cases 
appear  to  be  congenital. 

The  identity  of  retinitis  striata  and  angioid  streaks  remains  to  be 
established,  but  it  is  at  least  probable  that  both  conditions  originate 
from  blood  extravasated  into  the  retina.  That  this  is  the  origin  of  angioid 
streaks  has  been  established  by  both  Holden  and  deSchweinitz,  who  have 
watched  the  transformation  of  blood  into  brown  striae.  Holden  assumes 
that  the  affection  called  retinitis  striata  like  that  called  angioid  streaks, 
arises  through  periphic  hemorrhages  diffused  in  a  linear  manner  through 
the  deep  layers  of  the  retina  where  they  undergo  various  sorts  of  metamor- 
phosis. Inasmuch  as  the  retinal  epithelium  is  not  disturbed,  the  only  other 
apparent  source  for  the  brown  pigment  is  blood.  Plange  ascribed  the 
striae  to  hemorrhages  which  excited  secondary  hyperplasia  of  Miiller's 
fibers.  Dunn  suggested  an  inflammatory  origin  but  observing  no  change 
in  the  streaks  after  prolonged  observation,  he  accepted  a  hemorrhagic 
origin  for  the  condition.  Walser  thought  that  retinal  striae  might  be  due 
to  colloid  excrescences,  intrauterine  hemorrhage  or,  what  he  regarded 
as  most  probable,  to  folds  in  the  retin?.  Pretori  believes  in  the  congenital 
origin  of  the  affection.  Striae  may  form  in  cured  detachments  of  the  retina, 
but  reattachment  is  a  much  more  infrequent  cause  for  retinal  striation  than 
is  generally  supposed.  While  there  is  little  doubt  of  the  hemorrhagic 
origin  of  retinitis  striata,  it  is  difficult  to  understand  why  blood  should 
assume  a  linear  form  in  the  retina.  True,  Schwalbe  and  His  have  de- 
scribed retinal  lymph  spaces  radial  with  the  disc,  but  the  existence  of  such 
channels  is  doubtful.  It  is  highly  probable  that  some  cases  of  retinal  striae 
may  originate  in  natal  hemorrhage  occurring  while  the  infant's  thorax  is 
compressed  in  the  parturient  canal   (compression,  or  traumatic  asphyxia). 

Stereogram  62.  Retinitis  Striata.  Left  eyeground  of  a  woman 
aged  23  years.  Vision  in  the  right  eye  is  20/20  and  in  the  left  eye  20/50; 
states  that  vision  in  the  left  eye  has  always  been  defective. 

Ophthalmoscopic  Picture:  Media  clear;  optic  nerve  and  retinal 
vessels  appear  normal;  the  distinguishing  feature  in  the  fundus  is  a  number 
of  white  and  brownish  stris,  situated  in  the  retina  behind  the  vessels  but  in 
no  way  connected  with  them.  The  striae  are  white,  although  in  many  places 
they  are  overlaid  or  bordered  by  brown  pigment.      Pwo  stripes  start  from 


MISCELLANEOUS    AFFECTIONS    OF    THE    RETINA.  215 

the  edge  of  the  disc,  one  of  which  proceeds  upward  and  the  other  down- 
ward. At  first  they  are  straight  and  brown,  but  soon  branch  and  exhibit 
an  underlying  white  structure.  These  striae  are  joined  by  others  of  a  yel- 
lowish-white color  and  free  from  pigment,  which  extend  diagonally  across 
the  retina. 

The  origin  of  the  streaks  in  this  retina  can  only  be  surmised,  but  I 
am  ciisposed  to  regard  them  as  the  residue  of  hemorrhages  which  occurred 
either  during  birth  or  in  early  infancy. 

Diagnosis.  In  retinitis  proliferans  the  new-formed  tissues  are  situated 
in  front  of  the  retinal  vessels,  while  in  retinitis  striata  they  are  behind 
them.  Retinal  streaks  due  to  reattachment  of  a  detached  retina  are  diag- 
nosticated only  by  an  undoubted  history  of  preceding  detachment  as  in 
Gorlitz's  case.  From  a  preconceived  but  mistaken  view  of  their  etiology, 
many  cases  of  retinitis  striata  have  been  wrongly  attributed  to  detachment. 
A  very  narrow,  branching  rupture  of  the  choroid  might  be  mistaken  for 
retinitis  striata,  but  may  be  distinguished  by  its  crescentic  form  and  by  its 
situation  which  is  concentric  to  the  outline  of  the  disc. 

Bibliography. 

Nagle,   Klin.  mom.  f.  .\ughn.,  1864,  p.  394. 

Schilling,   A.   f.   Auglm.,   XLIII,   1901.     Arch,  of  Oph.  XXXII,   190.3.  219. 

Gloor,  A.   f.   Aughn.  XXXV.    1S97.  328.     Klin.  m.   f.   .Aughn.,  XXXVI,   1898,   137. 

Plange,  A.  f.  Aughn.,  XXIII,  1S91,  78. 

Herman   Knapp,  A.  of  Oph..   XXI.  2S9. 

deSchweinitz,  Rep.   Am.  Oph.   Soc.   1896;    \.  of   Oph.,  XXV,  1896,  p.   439. 

Gorlitz,    Kl.    m.    f.    Aughn.,    XXXV,    1897,    361. 

Dunn,   A.    f.    Aughn..   XXXIV,   1897,   294. 

Stephenson,   Trans.   Oph.    Soc,   XII,   1892,   140. 

Holden,    Arch,   of   Oph.,   XXIV.   1895,   278. 

Walser,   A.   f.   Aughn.,   XXXI,   1895,   345. 

Pretori,  Beitrag,  f    A.  XXIV.,  3.  1898,  393. 

Schwalbe,  Lehrbuch  d.  Anat.,  d.  Auges,  1887,  99-111. 

Metastatic  Retinitis  (Endogenous  Ophthalmitis).  Infection  of  the 
/etina  is  caused  either  by  exogenous  bacteria  introduced  through  perfora- 
tions of  the  globe  or  by  endogenous  material  carried  to  the  eye  through 
the  blood  channels.  Exogenous  retinitis  does  not  come  within  the  scope  of 
this  work.  Metastatic  ophthalmitis  may  develop  in  the  course  of  puer- 
peral fever,  surgical  sepsis  or  other  microbic  diseases.  All  parts  of  the 
eye  may  be  attacked,  but  in  this  discussion  only  retinal  metastases  will  be 
considered  (see  metastatic  choroiditis).  Two  principal  forms  of  metas- 
tatic retinitis  are  recognized:     (1)    A  progressive,   inflammatory  process 


216  THE    FUNDUS    OCULI. 

which  terminates  either  in  purulent  panophthalmitis,  phthisis  bulbi  or  in- 
flammatory detachment  of  the  retina;  (2)  Septic  retinitis  of  Roth,  dis- 
tinguished by  white  spots  and  hemorrhages  into  the  retina  and  by  its  non- 
progressive character. 

The  progressive,  Inflammatory  type  of  metastatic  retinitis  commences 
in  the  retina,  although  the  ciliary  body  is  always  inv^olved.  The  condition 
develops  rapidly.  White  spots  and  hemorrhages  appear  in  the  retina, 
the  vitreous  becomes  turbid,  total  detachment  of  the  retina  quickly  develops 
and  within  twenty-four  hours  vision  is  abolished.  The  attack  may  be  ac- 
companied by  pain.  F  rom  this  point  one  or  two  courses  may  be  pursued, 
namelv,  suppuration  or  resolution.  Suppuration  is  indicated  by  edema  of 
the  lids  and  puruleiit  iridocyclitis  culminating  in  bulbar  abscess.  In  certain 
cases  the  contents  of  the  eye  break  down  and  form  pus.  Spontaneous  per- 
foration, however,  may  not  occur,  and  the  globe  may  remain  in  the  condition 
of  a  "  cold  abscess,"  subject  to  inflammatory  exacerbations.  In  cases  which 
terminate  bv  resolution,  the  detached  retina,  agglutinated  by  inflammatory 
exudates,  forms  a  yellowish-white,  immobile  mass  behind  the  lens.  Pro- 
liferative processes  may  develop  in  the  retina  and  new  blood  vessels  form 
on  its  surface,  in  which  eveat  it  closely  resembles  a  malignant  neoplasm 
and  hence  is  known  as  "  pseudoglioma  "  (Stergm.  77).  In  children  this 
condition  requires  to  be  differentiated  from  true  glioma  (page  286).  The 
intraocular  tension  is  diminished  in  proportion  to  the  destruction  wrought 
to  the  ciliary  processes.  In  many  cases  the  tension  remains  normal.  If 
iritic  adhesions  exist  sufficient  to  obstruct  the  outflow  of  aqueous,  secondary 
glaucoma  develops. 

Pathology.  Metastatic  retinitis  probably  is  always  caused  by  bac- 
terial emboli  in  the  capillaries,  the  results  varying  according  to  the  viru- 
lence of  the  micro-organism  or  degree  of  immunity  conferred  upon  the 
tissues.  Failure  to  demonstrate  bacteria  in  the  tissues  is  not  evidence 
against  the  bacterial  origin  of  the  process,  inasmuch  as  these  organisms 
may  disappear  in  the  last  stages.  Treacher  Collins  regards  the  retina  as 
the  usual  starting  place  of  metastatic  endophthalmitis,  but  difficulty  is  ex- 
perienced in  finding  an  example  limited  to  the  retina. 

In  the  following  case  the  uvea  did  not  appear  to  be  involved  in  the 
active  inflammatorv  process.* 

Right  eye  of  an  infant  9  months  of  age.  Measles  prevailed  in  the 
family  during  the  period  of  the  mother's  gestation  and  parturition.     The 


*Prom  the  service  of  Dr.  A.  E.  Davis,  N.  Y.  Post  Graduate  Hospital.     The  eye  was 
sent  to  me  for  examination. 


MISCELLANEOUS    AFFECTIONS    OF    THE    RETINA.  217 

mother  thinks  the  child  was  born  blind.  A  yellowish-white  mass  was  seen 
behind  the  lens  of  each  eye.  The  right  eye  was  enucleated.  Microscopic 
examination  showed  that  the  intraocular  mass  consisted  of  the  totally  de- 
tached retina  which  was  connected  by  plastic  adhesions  to  the  ciliary  body 
and  lens.  Aside  from  this  the  ciliary  body  contained  no  evidence  of  Inflam- 
matory action    (Fig.    117). 

Frequent  opportunities  occur  to  study  the  early  stages  of  acute  sup- 
purative retinitis  developing  after  exogenous  infection  from  perforating 
wounds  and  corneal  ulcers.  1  he  infection  usually  Is  Implanted  in  the  cil- 
iary body.  Leucocytes  appear  In  the  retina  first  at  the  ora  serrata  and  at 
about  the  same  time  around  the  optic  disc,  traveling  from  the  anterior 
part  of  the  eye  backward  along  the  surface  of  the  retina  and  also  through 
the  hyaloid  canal.  They  soon  Infiltrate  the  retina  and  ensheath  the  ves- 
sels, particularly  the  veins.  There  is  intense  Inflammatory  edema  of  the 
retina  which  Is  quickly  detached  by  the  accumulated  fluid.  The  effusion 
Is  rich  In  protcids,  which  are  precipitated  by  reagents  employed  to  harden 
the  eye  and  appear  under  the  microscope  either  as  granular  deposits, 
fibrinous  coagula  or  rounded  hyaloid  masses.  If  suppuration  does  not 
ensue,  these  exudates  may  pass  through  the  degenerative  processes  which 
terminate  In  fatty  or  calcareous  deposits.  From  the  retina  the  purulent 
process  passes  to  the  vitreous  and  choroid.  The  contents  of  the  eyeball 
may  become  necrotic  and  be  discharged  as  pus  or  undergo  organization 
and  atrophy.  The  metastatic  retinitis  of  meningitis  and  measles  tends  to 
terminate  In  organization  rather  than  necrosis.  If  the  retina  undergoes 
proliferative  inflammation,  collections  of  lymphoid  and  giant  cells  may  be 
found.  Axenfeld  states  that  1.3  per  cent  of  the  cases  which  do  not  sup- 
purate excite  sympathetic  Inflammation.  This  statement  is  Important  and 
should  be  either  confirmed  or  disproved. 

Septic  Retinitis  of  Roth.  Roth  first  reported  that  in  septic  con- 
ditions a  retinitis  occurred  which  bore  some  resemblance  to  that  observed 
in  albuminuria.  In  this  condition  the  exterior  of  the  eye  usually  appears 
normal.  Exceptlonallv,  there  are  hemorrhages  into  the  conjunctiva  and 
slight  chemosis.  Pupillary  reactions  are  normal;  the  media  are  clear;  the 
nerve  head  is  neither  reddened  nor  swollen;  round  and  oval  white  spots 
appear  In  the  posterior  zone,  distributed  about  the  disc.  These  spots 
rarely  appear  in  or  near  the  macula  or  anterior  to  the  equator,  nor  do 
they  follow  the  vessels  but  when  In  contact  with  the  latter  the  spot  lies 
in  front.  Thev  manifest  no  tendencv  to  enlarge  or  to  coalesce.  Aside  from 
the  spots  there  Is  no  opacity  of  the  retina.     Superficial  hemorrhages,  dis- 


218  THE    FUNDUS    OCULI. 

tributed  in  a  manner  similar  to  the  spots,  but  not  connected  with  them, 
usually  are  present.  Sometimes  the  hemorrhages  contain  white  centers, 
and  Lenhartz  has  observed  these  in  perfectly  fresh  extravasations.  White 
spots  may  occur  without  hemorrhage  or  hemorrhages  without  white  spots, 
either  being  sufficient  to  establish  a  diagnosis  of  septic  retinitis.  Litten 
thought  that  retinal  hemorrhage  was  the  immediate  precursor  of  death 
but  Wilbrand  and  Saenger  have  observed  it  weeks  before.  Metastatic 
ophthalmitis  of  all  types  occurs  in  one  eye  about  twice  as  frequently  as 
in  both. 

Pathology.  The  propriety  of  considering  Roth's  septic  retinitis  as 
only  a  variation  of  the  severe  type  of  metastatic  retinitis  is  not  generally 
conceded.  I  hat  it  is  caused  by  toxins  generated  in  remote  parts  of  t^e 
body  remains  to  be  proven.  In  the  meantime,  micro-organisms  have  been 
demonstrateci  in  the  lesions  and  retinal  blood  vessels  of  typical  cases. 
Axenfeld-Goh  report  a  fatal  case  of  ulcerative  endocarditis  with  septic 
retinitis.  Masses  of  pneumococci  were  found  in  the  recent  retinal  deposits, 
but  in  the  older  spots  the  cocci  were  degenerating  or  had  disappeared.  One 
small  retinal  vein  was  filled  with  masses  of  bacteria.  In  the  retina  these 
bacteria  possess  but  slight  virulence.  Wilbrand  and  Saenger  describe  a 
case  in  which  the  retinal  vessels  were  filled  with  streptococci.  Grunert 
reports  a  case  presenting  both  types  of  metastatic  retinitis.  In  the  right 
eye  was  a  severe  iridocyclitis  with  vitreous  abscess,  and  in  the  left  eye  a 
typical  septic  retinitis  of  Roth.  In  the  latter  case  the  vitreous  remained 
clear  and  the  appearance  of  the  spots  was  unchanged  at  the  time  of  death. 
Reports  upon  the  histologic  changes  found  in  Roth's  spots  indicate  their 
localized  character.  Slight  edema  and  loss  of  nuclear  staining  may  exist  in 
the  adjacent  retina.  In  fresh  cases  Grunert  found  the  spots  to  consist  of 
sharply  defined  accumulations  of  round  cells.  Wilbrand  and  Saenger  found 
them  to  be  composed  of  massed  leucocytes.  Nearly  all  reports  mention  the 
presence  of  so-called  "  ganglionic  nerve  fibers,"  which  may  be  regarded 
as  only  altered  leucocytes. 

Diagnosis.  The  ophthalmoscopic  diagnosis  between  Roth's  spots 
and  miliary  tubercles  in  the-choroid  is  difficult.  Tubercles  have  a  reddish  or 
yellow  color,  while  the  septic  nodules  are  white.  Retinal  hemorrhages  are 
more  common  in  sepsis  than  in  miliary  tuberculosis.  An  optic  neuritis 
would  suggest  cerebral  tubercle.  The  solitary  choroidal  tubercle  is  much 
larger  than  Roth's  s"ots. 


MISCELLANEOUS    AFFECTIONS    OF    THE    RETINA.  219 

R'lbiiogrciphy. 

Axenfeld,  A.   f.  O.  Oph.  XL,  4,   1,S94. 

Goh.  A.  f.  O.  XLIII,  L  1897. 

Gninert.   Bericht  d.   Heid.   Oph.   Gesl.   1903,   3.3S. 

Lenhartz,   Sp.   Path.   u.   Therp.   v.   Nothnagle,   Bd.   TIT,   T.  2,   192. 

Littcn,   Bercht.   d.    Heid.   Oph.   Gesl.   1877,   140.  , 

Roth,  A.  f.  Oph.  LV.  1873.    Deutsch.  z.  f.  Chir.  I,  1873,  471. 

Treacher   Collins. 

Wilbraiul,   u.   Saenger.   vol.   IV,   1909,  354. 


Chapter  XL 
OPTIC  NEURITIS  AND  OPTIC  ATROPHY. 

Optic  Neuritis.  The  study  of  optic  neuritis  will  be  facilitated  by 
recognizing  two  pathologic  conditions  which  may  or  may  not  merge  into 
each  other.  These  are,  (I)  papilledema  or  passive,  noninflammatory 
swelling  of  the  nerve  head,  and  (2)  true  neuritis  or  active  inflammation 
of  the  nerve,  situated  either  behind  the  globe  (retrobulbar  neuritis)  or  at 
the  ocular  end  intraocular  neuritis;  papillitis).  The  term  "  choked  disc  " 
has  been  arbitrarily  applied  to  any  swelling  of  the  nerve  head  which  reaches 
an  elevation  of  1  mm.  (3.D.)  although  strictly  speaking  it  should  be 
reserved  for  cases  of  noninflammatory  edema  or  papilledema.  Never- 
theless the  term  as  now  employed  is  clinically  convenient. 

Two  theories  are  prominent  as  to  the  pathogenesis  of  choked  disc, 
both  of  which  recognize  the  important  anatomic  fact  that  cerebrospinal 
fluid  passes  directlv  from  the  cranial  cavity  into  the  sheaths  of  the  optic 
nerves:  (  1  )1  he  Manz,  Schmidt-Rimpler  theory,  according  to  which  a  high 
intracranial  pressure  retards  the  return  lymph  current  from  the  nerve 
sheaths  to  the  brain.  This  causes  distension  of  the  sheaths  and  forces 
fluid  into  the  ocular  end  of  the  nerve  and  into  the  rigid  lamina  cribrosa, 
thus  strangulating  the  retinal  vessels  and  the  intraocular  portion  of  the 
nerve  (Fig.  139).  Parinaud  agrees  with  this  theory  as  regards  lymph  ob- 
struction, but  believes  that  it  acts,  not  through  ciistension  of  the  sheath  but 
by  causing  edema  of  the  entire  nerve  trunk  including  the  intraocular  end 
(Figs.  140-141).  (2)  The  inflammatory  theory  of  Leber  assumes  that 
intracranial  disease  prociuces  toxins  which  are  carried  by  lymph  currents 
into  the  optic  sheaths  where  they  excite  inflammatory  reaction  in  the  nerve 
and  disc. 

Both  the  above  theories  are  measurably  true,  but  each  is  applicable 
to  a  different  form  of  neuritis.  In  choked  disc  produced  experimentally 
and  by  brain  tumor,  the  microscope  fails  to  reveal  evidence  of  true  inflam- 
mation, although,  if  swelling  of  the  nerve  head  is  excessive,  there  may  be 
local  tissue  reaction  with  round  celled  inhltration  as  occurs  in  the  analogous 
condition  of  strangulated  hernia.     On  the  other  hand,  in  the  metastatic  and 

220 


Fig.  139— Choked  Disc.  The  Sheaths 
of  the  Optic  Nerve  are  Distended  with 
Fluid  Causing  Strangulation  of  the 
Retinal  Vessels  and  Intraocular  Por- 
tion of  the  Nerve  (Manz,  Schmidt- 
Rimpler  theory). 


Fig.  VM. 


Fig.  140 — Choked  Disc  showing  Edema 
of  entire  Xerve  Trunk,  including  Intra- 
ocular end. 


Fig.  140. 


Fig.  141 — Choked  Disc.  Edema 
of  entire  Nerve  Trunk.  Similar  to 
Fig.    140. 


Fig.  141. 


Fig.  142. 


Fig.  143. 


Fig.  142 — Papilledema,  or  Choked  Disc.     The  Excessive  Swelling  of  the  Nerve  Head 
is  Non-infiammatory  and  due  to  Passive  Edema. 

Fig.    143 — Papilledema,    or   Choked    Disc.     The    same   conditions   exist   as   in   Fig.   142. 


Fig.  1-;^. 

Fig.  144 — Choked  Disc  Following  Injury 
to  the  Cranium. 


FiK.  u:, 

Fig.    143  —  Optic     Neuritis    in    Cerebral 
Tuberculosis. 


OPTIC    XEURiriS    AND    OPTIC    ATROPHY.  221 

descending  neuritis  of  septic  and  infectious  diseases,  in  syphilis,  tubercu- 
losis, sinus  thrombosis,  etc.,  inflammatory  infiltration  of  the  nerve  is 
marked  although,  if  only  the  proximal  end  of  the  nerve  is  involved,  the 
disc  may  not  be  swollen.  The  greatest  swelling  of  the  nerve  head 
observable  with  the  ophthalmoscope  occurs  in  papilledema,  which  is 
a  noninflammatory  condition  due  only  to  passive  congestion  and  edema 
(Figs.    142-143). 

In  the  great  majority  of  cases  choked  disc  is  obviously  due  to  me- 
chanical compression  of  the  cranial  contents  by  tumors,  inflammatory  prod- 
ucts, depressed  bone,  excess  of  cerebrospinal  fluid,  blood,  etc.  It  appears 
to  be  very  common  after  mastoid  operations  involving  the  jugular  vein 
or  lateral  sinus  (Stergm.  66).  Exceptionally,  choked  disc  is  due  to  menin- 
gitis, and  to  infectious  and  constitutional  disease,  especially  syphilis;  also 
to  injury  (Fig.  144),  exposure,  sunstroke,  nasal  sinus  disease,  caisson 
disease,  arteriosclerosis  and  certain  poisons.  Other  cases  appear  to  be 
hereditary.  Very  rarely  choked  disc  accompanies  myelitis,  disseminated 
sclerosis  and  general  paresis.  In  children,  optic  neuritis  usually  depends 
on  chronic  meningitis  or  cerebral  tuberculosis  (Fig.  145).  Choked  disc 
is  very  rare  in  hydrocephalus  externus  but  is  common  in  acute  hydrocephalus 
internus  (serous  meningitis)  in  adults  except  in  those  cases  attended  by 
nasal  discharge  of  cerebrospinal  fluid.  Serous  meningitis  seldom  causes 
choked  disc  in  children  in  whom  excessive  brain  pressure  is  relieved  by  sep- 
aration of  the  ununited  cranial  bones.  Uthoff,  in  100  cases  of  syphilis  of 
the  nervous  system,  found  choked  disc  or  optic  atrophy  in  40  per  cent.  The 
syphilitic  changes  consisted  of  gummatous  new  formations,  65  per  cent; 
gummatous  meningitis  (usually  basilar),  23  per  cent;  v^ascular  disease,  8 
per  cent;  syphilitic  hydrocephalus  internus  2  per  cent;  double  periostitis  of 
orbit  2  per  cent.  In  cerebral  hemorrhage  3  p^  cent  of  the  cases  develop 
choked  disc  which,  according  to  Uthoff,  is  always  due  to  percolation  of 
blood  from  the  cranial  cavity  into  the  nerve  sheath  (hematoma  of  the 
sheath).  The  infrequency  of  choked  disc  in  cerebral  hemorrhage  may 
be  explained  either  by  rapid  recession  of  high  brain  pressure  or  by  quick 
death  of  the  subject. 

The  full  development  of  a  choked  disc  (papilledema)  may  occupy 
weeks  or  months.  Rapid  development  accompanied  by  early  loss  of  vision 
indicates  an  inflammatory  or  toxic  condition  (true  neuritis).  It  is  not 
unusual  in  papilledema  for  vision  to  remain  normal  for  months,  showing 
that  the  nerve  fibers  have  not  been  constricted  sufficiently  to  destroy  their 
conductivity.     Usually,  however,  vision  is  blunted  and  unless  pressure  is 


222  THE    FUNDUS    OCULI. 

relieved  it  is  permanently  destroyed.  In  brain  tumor,  after  full  develop- 
ment of  papilledema,  little  ophthalmoscopic  change  occurs  for  a  long 
time;  months  or  even  a  year  may  pass  when,  gradually,  the  swelling  sub- 
sides, the  outlines  of  the  disc  reappear  and  atrophy  is  established. 

Stereogram  63.  Papilledema  (choked  disc)  in  Brain  Tumor. 
Right  eyeground  of  a  man  aged  37  years.  Has  double  choked  discs  due 
to  brain  tumor.  The  fellow  eye  is  shown  in  stereogram  64.  The  head  of 
the  optic  nerve  forms  a  mound-like  projection  rising  1.3  mm.  (5.D.) 
above  the  level  of  the  fundus.  The  diameter  of  the  disc  apparently  is 
increased  owing  to  extension  of  edema  from  the  nerve  into  the  neighboring 
retina.  The  swollen  tissue  of  the  nerve  has  compressed  the  retinal  vessels 
and  retarded  circulation.  The  arteries  are  straight  and  narrowed  but, 
evidently,  are  conveying  more  blood  to  the  retina  than  the  obstructed  veins 
can  carry  away.  As  a  result,  the  veins  are  darkened,  tortuous  and  dilated. 
Tortuosity  is  extreme  in  the  inferior  macular  vein  which  twists  like  a  cork- 
screw, and  also  in  a  vertically  ascending  vein  the  convolutions  of  which  rise 
and  fall  in  a  plane  perpendicular  to  the  retina.  No  hemorrhages  are  seen 
but  serous  transudation  (retinal  edema)  has  occurred  along  some  of  the 
vessels. 

Coloring  for  a  choked  disc  is  supplied  by  a  dark  red.  Intensely  hyper- 
emic  nerve  head  overlaid  by  translucent,  edematous  nerve  fibers.  In  this 
nerve  the  swelling  is  moderate  in  amount,  the  disc  has  a  pale  rose  color 
and  its  surface  is  delicately  veined  by  the  engorged  capillaries.  In  cases 
attended  bv  excessiv^e  swelling,  the  serous  infiltrates  may  completely  obscure 
the  red  disc  and  the  nerve  head  will  be  very  light  in  color.  The  light  spot 
observed  in  the  center  of  this  disc  is  a  deep  phvsiologic  excavation  filled 
bv  edematous  tissue.  Despite  the  alarming  appearance  of  the  fundus,  vision 
is  normal. 

Diagnosis.  The  broad,  light  colored,  elevated  surface  exhibited  by 
this  disc  could  be  nothing  but  optic  neuritis.  In  the  absence  of  hemorrhage, 
venous  thrombosis  would  not  be  considered  in  the  differential  diagnosis. 
There  are  no  ophthalmoscopic  signs  which  distinguish  the  choked  disc  of 
brain  tumor  from  that  caused  by  other  conditions,  but  in  this  case  the 
absence  of  vascular  degeneration,  hemorrhage  and  plastic  exudation  tends 
to  exclude  all  forms  of  anglopathic  neuritis  and  Indicates  a  cerebral  origin 
for  the  swelling. 

In  all  cases  of  true  papilledema  it  Is  probable  that  intracranial  pres- 
sure is  relatively  high.  The  deleterious  effect  which  this  exerts  upon  the 
optic  discs  depends  upon  the  unyielding  bony  walls  of  the  cranial  cavity 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  223 

which  vent  excessive  internal  pressure,  through  the  optic  foramen  and 
into  the  intervaginal  space  around  the  optic  nerve.  This  is  verified  in 
cases  of  brain  tumor  where  choked  discs  disappear  after  intracranial  pres- 
sure is  relieved  by  trephining  the  skull. 

In  the  great  majority  of  cases  choked  disc  is  caused  by  tumor  of  the 
brain,  and  of  all  the  symptoms  which  brain  tumor  presents,  choked  disc  is 
most  nearly  pathognomonic.  The  ophthalmologist  is  required  not  only 
to  discover  choked  disc  but  to  ascertain  its  origin;  therefore,  he  should 
possess  a  knowledge  of  its  principal  cause.  In  brain  tumor  papilledema 
develops  in  about  80  per  cent  of  all  cases.  It  is  said  to  occur  most  fre- 
quently with  cysts  and  glioma,  less  often  with  tuberculous  growths.  The 
nature  of  tubercle  is  to  replace,  not  displace  tissue;  therefore,  it  is  possible 
for  a  tuberculoma  to  develop  in  the  brain  without  increasing  the  cranial 
content.  I'he  location  of  a  tumor  is  more  influential  in  causing  choked 
disc  than  its  size;  thus,  it  is  almost  constant  in  tumors  of  the  cerebellum 
and  corpora  quadragemini,  but  less  frequent  in  tumors  of  the  corpus  cal- 
losum  and  convexity.  Choked  disc  from  brain  tumor  usually  is  bilateral, 
but  the  degree  of  swelling  or  stage  of  progress  may  vary  widely  in  the  two 
eyes.  When  unilateral,  it  may  occur  in  either  eye,  but  is  most  frequent  on 
the  side  of  the  tumor.  Tumors  situated  far  forward  in  the  brain  are  more 
apt  to  cause  choked  disc  on  the  same  side  than  when  situated  far  back,  but 
this  is  not  always  the  case.  In  unilateral  papilledema  the  possibility  cf  a 
growth  which  involves  one  nerve  must  be  borne  in  mind. 

The  most  frequent  general  symptoms  of  brain  tumor  are  headache, 
slow  pulse,  expulsive  vomiting,  epileptic  seizures,  apoplectic  attacks  and 
psychic  disturbances.  The  focal  symptoms  are  perversion  or  loss  of  func- 
tion in  a  spinal,  cranial  or  special  nerve.  There  may  be  loss  of  vision  witli- 
out  eye  lesion,  disturbance  of  speech,  inequality  of  pupils,  tremors,  localized 
spasms,  choreiform  movements,  paralysis  or  contractures.  Focal  symptoms 
to  be  diagnostic  of  brain  tumor  must  be  unilateral  and  not  suddenly  de- 
veloped. 7  ransitory  attacks  of  blindness  are  a  highly  significant  symptom. 
Gushing,  in  12.3  cases  of  brain  tumor,  found  interlacing  or  inversion  of 
the  color  fields  Myschromotopsia)  in  43  per  cent.  The  cortical  centers 
of  vision  are  in  the  occipital  lobes;  consequently,  tumors  of  this  region  are 
rich  in  morbid  visual  phenomena  which  strongly  suggest,  but  do  not  prove, 
occipital  disease.  These  consist  of  hemianopsia,  hemianopsia  for  colors 
with  normal  fields  for  white,  photopsis  and  visual  hallucinations.  Mind 
blindness  indicates  a  lesion  of  the  occipital  lobes.     Tumors  of  the  hypo- 


224  THE    FUNDUS    OCULI. 

physis,  as  in  acromegaly,  press  on  the  optic  chiasm  and  produce  bitemporal 
hemianopsia.  The  same  phenomenon  may  be  due  to  hyperdistension  of  the 
third  ventricle. 

Except  in  syphilitic  cases,  which  may  improve  under  treatment,  the 
course  of  brain  tumor  is  steadily  progressive. 

Stereogram  64.  Papilledema  (Choked  Disc)  in  Brain  Tumor. 
Left  eyeground  of  a  man  37  years  of  age.  Has  brain  tumor  with  bilateral 
choked  discs.  The  right  eye  is  shown  in  stereogram  63.  In  the  left  eye 
the  papilledema  is  far  more  intense  than  in  the  right.  The  nerve  head 
forms  an  abrupt  projection  2.5  mm.  (7.5  D. )  above  the  level  of  the  fundus.* 
The  swelling  of  the  nerve  offers  great  obstruction  to  the  return  circulation, 
as  shown  by  the  unusual  number  of  hemorrhages  and  by  the  retinal  edema 
which  is  more  extensive  than  commonly  observed  in  choked  disc.  The 
arteries  are  small  and  straight.  At  the  periphery  of  the  fundus  the  veins 
form  long  sinuosities,  but  toward  the  disc  they  become  extremely  tortuous 
and  dip,  with  short  vertical  curves,  into  the  edematous  retina  until,  almost 
bursting  with  blood,  they  force  their  way  into  the  swollen  nerve  head.  The 
edematous  retina  around  the  nerve  presents  delicate,  radial  striations 
which  conform  to  the  course  of  the  nerve  fibers.  The  striate,  feathery 
hemorrhages  at  the  border  of  the  disc  and  elsewhei'e  are  situated  in  the 
nerve  fiber  layer,  while  the  large,  dark,  rounded  extravasations  are  in  the 
deeper  layers  of  the  retina.  Vision  in  this  eye  is  reduced  to  10/20'9.  The 
deterioration  is  ascribed  partly  to  degenerative  changes  in  the  nerve  fibers 
and  partly  to  hemorrhages  in  the  macular  region. 

Diagnosis.  The  enormous  swelling  of  this  nerve  head  constitutes 
an  unmistakable  choked  disc.  The  question  of  associated  retinal  disease 
always  arises  in  cases  of  optic  neuritis  and  its  presence  or  absence  modifies 
the  diagnosis  of  the  underlying  condition.  Although,  in  this  case,  hemor- 
rhage is  abundant,  it  is  far  less  than  occurs  in  thrombosis  of  the  central 
vein  (Stergm.  46)  ;  moreover,  neuritis  usually  is  absent  in  thrombosis  and 
when  present  is  mild  in  character.  In  albuminuric  retinitis  (Stergms. 
35-36)  the  exudates,  transudates  and  hemorrhages  occur  independently 
of  nerve  disease  or  vascular  constriction,  while  in  choked  disc  they  are  pro- 
portionate to  the  degree  of  nerve  swelling.  The  cause  of  a  choked  disc  is 
not  ascertained  from  the  ophthalmoscopic  picture,  but  from  an  investigation 
of  the  systemic  condition. 


*The  height  of  a  choked  disc  is  measured  by  the  difference  in  refracti m  between  the 
normal  fundus  and  apex  of  the  nerve. 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  225 

Bibliography. 

Gushing,  John   Hopkins   Hosp.   Bui.,  June   1911.     Rep.   Section  Oph.   Am.   Med.   Assn. 

1911,  p.   132. 
Uthoff,  Graefe-Saemisch   Handb.  2.  p.   1043. 

Manz.  Zehcndcr's   .Monatshl..   Ikl.    Ill,   1865.   Arch.   f.  O.  XVI.   1,  265. 
Schmidt,  Arch.   f.  O..  XV,   1S69,   193. 

Leber,  Graefe   Saemisrh   Handb,   Bd.   V,   1877.    Trans.    Int.   Gong.   1881. 
Parinkud.   N.  &  Oliver,   vol.   HI,  p.  600. 

Neuritis  in  N.\sal  Sinus  Disease.  Discovery  of  the  relation  be- 
tween diseases  of  the  accessory  nasal  sinuses  and  diseases  of  the  eye 
marked  an  important  advance  in  medical  knowledge.  While  little  is  known 
of  the  anatomic,  histologic  or  bacterial  changes  which  involve  the  eye  in 
nasal  disease,  a  mass  of  clinical  data  of  diagnostic  value  has  accumulated. 

The  accessory  sinuses  consist  of  the  maxillary  antrum,  frontal  sinus, 
ethmoidal  sinus  and  sphenoidal  sinus.  These  cavities  frequently  are  the 
sent  of  acute  and  chronic  inflammation,  and  as  they  are  in  contact  with 
the  orbit  and  optic  nerve  {V\g.  146),  the  eye  usually  is  more  or  less  dis- 
turbed. The  inflammatory  processes  that  occur  within  the  cells  of  the 
sinuses  consist  of  acute  and  chronic  suppuration,  chronic  thickening  and 
polypoid  degeneration  of  the  mucous  membrane,  causing  distension  of  the 
n.vities  and  circulatory  disturbance.  In  neglected  cases,  caries  and  necrosis 
of  the  bony  walls  ensue.  The  serious  complications  are  abscess  of  the  orbit, 
basilar  meningitis,  intradural  and  extradural  abscess  and  thrombosis  of  the 
cavernous  sinus.  The  eye  is  affected  chiefly  through  the  optic  nerve.  The 
nerve  is  intimately  connected  with  the  cells  of  the  sphenoid  and  ethmoid, 
but  owing  to  wide  anatomic  variations,  the  relation  between  nerve  and 
sinuses  is  inconstant.  For  example,  the  nerve  may  be  separated  from  the 
sinuses  by  either  thick  or  thin  plates  of  bone:  sometimes  it  is  enclosed 
within  the  sinus  walls  or  the  optic  canal  may  run  entirely  within  the  sphe- 
noidal or  ethmoidal  cells.  The  method  by  which  sinus  disease  affects  the 
optic  nerve  has  not  been  ascertained,  but  may  be  explained  either  by  pres- 
sure on  the  nerve,  pressure  on  the  nutrient  vessels,  local  edema  or  through 
the  action  of  toxins.  In  but  few  cases  has  infection  appeared  to  reach  the 
nerve  by  continuity.  Necrosis  and  perforation  of  bone  are  not  essential 
to  extension  of  an  infectious  process  from  the  accessory  sinuses  to  the 
brain,  inasmuch  as  infection  can  be  conveyed  through  the  venous  anasto- 
moses that  exist  between  the  mucous  membrane  and  dura  mater.  A 
neuritis  may  develop  from  secondary  disease  of  the  brain,  presenting 
symptoms  which   vary   from   those   of  uncomplicated   nasal   disease.      In 

13 


226  THE    FUNDUS    OCULI. 

most  cases  neuritis  is  unilateral  when  due  to  accessory  sinus  disease,  and 
bilateral  when  due  to  brain  disease. 

Symptoms.  Owing  to  the  close  relationship  of  the  cavities  an  in- 
flammatory process  often  is  diffuse;  special  symptoms,  however,  may  in- 
dicate localization  of  disease  in  certain  cavities. 

Acute  inflammation  of  the  antrum  of  Highmore  may  be  attended  by 
pain  about  the  orbit,  lachrymation,  unilateral  purulent  discharge  from 
the  nose  and,  sometimes,  disturbed  vision.  In  many  cases,  however, 
these  symptoms  are  but  slightly  developed.  If  the  antrum  is  occupied  by  a 
cyst  it  may  be  so  distended  as  to  encroach  upon  the  orbit  but  seldom  to 
the  same  extent  as  the  other  sinuses.  When  the  antrum  is  occupied  by  fluid 
or  tumors  it  does  not  transilluminate.  Opacity  can  be  demonstrated  also 
by  means  of  the  X  ray.  Positive  evidence  of  pus  or  exudation  in  the  antrum 
is  obtained  by  puncture. 

Frontal  Sinus  Disease  is  attended  by  severe  periodic,  morning 
headache  and  tenderness  of  the  supraorbital  and  infraorbital  nerves.  The 
anterior  and  nasal  walls  of  the  orbit  are  tender  to  pressure.  Eventually, 
some  part  of  the  sinus  wall  is  apt  to  bulge  into  the  orbit,  usually  the  upper 
inner  angle.  As  in  antrum  disease,  both  the  transilluminator  and  X  ray  may 
be  emploved  to  discover  opacity  in  the  sinus. 

Disease  of  the  etJimoid  also  may  produce  a  cvstic  tumor  which,  unless 
relieved  by  operation,  may  open  through  the  skin  and  form  a  chronic  fistula; 
or  it  may  perforate  into  the  orbit,  the  antrum  or  the  nasal  cavitv-  Eye 
symptoms  often  appear  in  disease  of  the  ethmoid. 

The  sphenoidal  cells  frequently  are  affected  with  the  ethmoidal. 

The  close  connection  of  the  optic  nerve  with  the  ethmoid  and  sphe- 
noid in  more  than  40  per  cent  of  posterior  sinus  inflammations  results  in 
visual  disturbance.  The  anatomic  variation  of  these  structures,  however, 
is  so  great  that  eye  phenomena  are  of  little  value  in  localizing  the  seat  of 
disease,  except  that  in  posterior  sinusitis  eye  disturbance  is  very  common 
and  orbital  cellulitis  is  rare,  while  in  frontal  sinus  disease  this  order  of 
frequency  is  reversed.  The  X  ray  is  employed  to  photograph  the  ac- 
cessory sinuses  and  when  skilfully  performed  the  skiagraphs  aftord  val- 
uable information  regarding  the  presence  of  exudates  in  the  cells.  An 
occasional  early  symptom  of  sinus  disease  is  edema  of  the  lids.  Fre- 
quently, this  is  of  an  ephemeral,  recurrent  character.  It  may  consist  only 
of  slight  pufl'iness  most  marked  on  the  nasal  side  of  the  upper  lid,  or  the 
entire  lid  may  be  swollen.  Hard  or  soft  swelling  along  the  orbital  ring, 
due  to   a  low  grade   periostitis,  is  common   in  chronic   sinusitis.      If  the 


L.Txowtrt^  Sinus. 
L..  AwttYvov  ^tV\mo  vcL.  ' 


T^.TranlaV  Su\os. 


Fig.  146. 


Z.vT. 

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OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  227 

distended  cells  encroach  upon  the  orbit,  the  eye  may  be  displaced  and  the 
patient  experience  diplopia.  Paralysis  of  the  extrinsic  ocular  muscles  may 
occur  in  both  acute  and  chronic  sinus  disease,  but  is  more  common  in  the 
acute  inflammatory  type.  Pain  may  be  experienced  in  extreme  upward 
excursion  of  the  eye.  1  he  effect  of  nasal  sinus  disease  upon  the  optic 
nerve  is  of  the  greatest  value  in  determining  the  nature  of  obscure  cases. 
As  a  rule,  onlv  one  eye  is  affected.  Bilateral  affection  of  the  eyes,  espe- 
cially swelling  of  the  discs,  suggests  intracranial  complications.  Changes  in 
the  optic  disc  usually  are  not  m?rked.  They  consist  of  slight  marginal  ob- 
scuration, on  the  nasal  side,  fullness  of  the  ^'eins  and  visible  peri\ascular 
lymph  channels  on  and  near  the  nerve.  Even  these  alterations  are  present 
in  onlv  a  minority  of  cases.  Occasionally,  the  nerve  head  is  swollen  and 
a  mild  bulbar  neuritis  is  established.  The  subjective  symptom.s  indicate  a 
retrobulbar  neuritis,  \earlv  all  proved  cases  show  marked  contraction  of 
the  color  fields  which  may  interlace.  There  is  relative  central  scotoma 
for  colors  which  in  advanced  cases  may  become  an  absolute  scotoma.  The 
normal  blind  spot  is  enlarged.  The  field'  for  form  is  seldom  contracted 
in  the  early  stages  (Fig.  147).  While  these  findings  are  applicable  to 
the  majority  of  cases,  the  most  varied  visual  phenomena  as  hemianopsia, 
ring  scotoma,  etc.,  may  de\elop.  \  ision  may  be  slightlv  reduced  or  it 
may  be  completelv  abolished.  Unless  the  condition  is  promptlv  relieved 
by  operation  on  the  sinuses,   optic  atrophy  ensues. 

The  prognosis  as  regards  vision  depends,  in  a  measure,  upon  the 
degree  of  deterioration  that  exists  at  the  time  of  operation,  but 
chieflv  upon  the  duration  of  the  process.  If  optic  atrophy  is  already 
established  the  outlook  is  unfavorable.  Harmon  Smith  got  the  best 
visual  results  from  operation  in  those  cases  where  the  sinuses  were  not 
markedlv  diseased.* 

Stereogram  65.  Right  eyeground  of  a  woman,  50  years  of  age. 
Since  an  attack  of  influenza  six  months  ago  she  has  suffered  from  head- 
ache. The  right  eye  appears  normal.  Left  eye,  vision  20/100.  ^"isual 
fields  show  marked  contraction  for  red  and  green  with  normal  field  for  form 
(white).  There  is  mild  optic  neuritis.  The  disc  is  elevated  about  0.5 
mm.  (1.5  D. )  ;  on  the  nasal  side  the  outline  is  obscure  and  the  adjacent 
retina  is  edematous.  On  and  near  the  disc  the  vessels  are  bordered  by 
white  lines  which  are  supposed  to  be  dilated  perivascular  sheaths.  All 
veins  are  somewhat  dilated  and  one,  the  superior  nasal,  is  quite  tortuous. 
The  arteries  are  straight. 


*Manhattan  Eye.  Ear  and  Throat  Hospital   Reports,  1912. 


228  THE    FUNDUS    OCULI. 

The  patient  was  referred  to  the  rhinologic  department  of  the  hospital 
where  a  diagnosis  was  made  of  posterior  accessory  sinus  disease.  Opera- 
tion by  Dr.  L.  Coffin  was  followed  by  relief  of  pain,  but  vision  did  not 
improve  and  when  last  seen  the  optic  nerve  was  atrophic. 

Diagnosis.  Moderate,  unilateral  swelling  of  the  optic  disc  should 
always  suggest  disease  oi  the  accessory  nasal  sinuses.  Neuritis  of  nasal 
origin  is  further  distinguished  by  the  subjective  symptoms  of  retrobulbar 
neuritis,  i.  e.,  relative  central  scotoma  for  colors  and  marked  contraction 
of  the  fields  for  red  and  green.  The  diagnosis  should  be  confirmed  by 
a   rhinologic   examination. 

The  Optic  Nerve  in  Cerebral  Sinus  Thrombosis.  Choked  disc 
and  optic  neuritis  are  common  events  in  mastoiditis  associated  with  sinus 
thrombosis  or  other  intracranial  complication,  Ruttin,  in  30  cases  of 
lateral  sinus  and  jugular  operations  found  8  in  which  the  optic  nerve  was 
affected.  This  appears  to  be  a  high  percentage.  J.  D.  Richards  examined 
100  similar  cases  and  found  choked  discs  in  about  10  per  cent.  The 
papilledema  or  neuritis  is  bilateral  in  more  than  half  the  cases;  when 
unilateral  it  usually  occurs  on  the  same  side  as  the  operation.  Probably, 
the  proportion  of  bilateral  cases  would  be  increased  were  the  conditions 
which  produce  choked  disc  prolonged.  In  surgical  cases  the  period  at 
which  papilledema  develops  varies  from  two  days  to  three  months  after 
operation  (Ruttin).  The  majority  of  cases  appear  to  be  simple  choked 
disc  due  to  heightened  intracranial  pressure  or  local  lymph  stasis  in  the 
nerve.  Others,  however,  present  the  symptoms  of  focal  inflammation 
in  the  nerve  itself,  /'.  c,  retrobulbar  or  intrahulbar  neuritis. 

The  mechanism  by_wh'ch  otitic  disease  produces  papilledema  has  not 
been  determined.  Many  regard  thrombosis  of  the  cavernous  sinus  as 
the  immeciiate  cause,  for  the  reason  that  frequently  it  is  associated  with 
choked  disc,  and  also  because  the  return  blood  from  the  eye  is  dischargeci 
into  this  sinus  by  the  ophthalmic  vein.  As  a  matter  of  fact,  obstruction  in 
the  cavernous  sinus  does  not  permanently  affect  intraocular  circulation 
owing  to  the  free  anastomoses  which  the  ophthalmic  vein  has  with  both 
the  facial  and  pterygo-palatine  veins.  Moreover,  complete  obstruction 
of  the  retinal  venous  circulation  does  not  cause  choked  disc  but  does  cause 
retinal  "  apoplexy"  (Stergm.  46).  Cavernous  sinus  thrombosis  can  exert 
no  direct  influence  upon  the  lymph  current  in  the  nerve  sheaths,  although  it 
may  cause  obstruction  by  inducing  collateral  edema.  Uthoff  states  that 
one  half  the  cases  of  cavernous  sinus  thrombosis  show  no  changes  in  the 
nerve.      Bartels    states    that    a    postoperative    choked    disc    is  proof  that 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  229 

the  intracranial  space  has  been  diminished.  Undoubtedly,  many  cases 
are  due  to  heightened  intracranial  pressure  produced  by  effusion  of 
serum.  Uthoff  considers  that  choked  disc  is  due  in  lateral  sinus 
thrombosis  to  wide  ramifications  of  the  clot  which  extend  into  the 
veins  from  the  surface  causing  meningeal  edema,  and  into  the  vena  Galeni, 
causing  hydrops  of  the  ventricles.  Bartels  likewise  considers  that  nearly 
all  cases  of  postoperative  papilledema  depend  upon  serous  meningitis  and 
hydrocephalus  internus,  and  when  the  edema  subsides  the  nerve  swelling 
disappears.  He  does  not  regard  sepsis  alone  as  causative  and  cites  a  case 
which  de\Tloped  sepsis,  nephritis  and  choked  disc.  1  he  choked  disc  soon 
disappeared,  while  the  other  conditions  persisted,  terminating  in  endocar- 
ditis and  death.  Septic  thrombosis  which  extends  into  the  orbit  does  not 
always  produce  choked  disc,  as  shown  by  another  case  of  Bartels  in  which 
purulent  thrombosis  of  both  cavernous  sinuses  extended  into  the  orbits 
causing  bilateral  phlegmon,  but  the  optic  nerves  were  not  affected  until  late 
in  the  disease  when  pachymeningitis  and  high  brain  pressure  developed.  In 
this  case  both  cavernous  sinuses  were  absolutely  closed,  nevertheless  the 
retinal  veins  were  not  congested,  showing  the  perfect  relief  which  the 
normal  venous  anastomoses  afford  the  ocular  circulation.  Another  similar 
case  showed  no  choked  disc,  although  a  clot  extendeci  far  into  the  oph- 
thalmic vein.  Bartels  discusses  another  class  of  cases  in  which  the  wound 
progresses  favorably  and  no  Intracranial  complications  are  demonstrable, 
yet  the  disc  becomes  choked. 

The  development  of  choked  disc  in  postoperative  thrombosis  does 
not  necessarily  mean  that  the  general  condition  of  the  patient  is  worse, 
in  fact,  it  Is  of  little  prognostic  import.  Thus,  it  may  indicate  an  increase 
in  intracranial  pressure  from  extension  of  an  abscess  or  from  simple 
edema.  In  other  cases  neuritis  may  be  caused  by  either  local  inflammation 
of  the  nerve,  extension  of  meningitis  to  the  nerve  sheaths  or  obstruction 
of  the  internal  optic  foramen  by  edema,  pus,  exudates,  etc. 

Intrabulbar  and  retrobulbar  neuritis  not  attributable  to  heightened 
intracranial  pressure  may  occur  in  postoperative  thrombosis.  In  such 
cases  the  neuritis  is  to  be  attributed  to  complications.  The  following  ex- 
ample of  this  condition  occurred  in  the  ear  service  of  Dr.  J.  F.  McKernon. 

Woman,  22  years  of  age.  Right  postoperative  septic  thrombosis. 
The  lateral  sinus  was  opened  and  the  jugular  was  resected.  Nineteen 
days  after  operation  menmgitis  developed.  Three  days  later  she  became 
blind  with  widely  dilated  pupils.  The  right  optic  nerve  gradually  became 
choked,  reaching  an  elevation  of  nearly  2  mm.     Vision  in  this  eye  began 


230  THE    FUNDUS    OCULI. 

to  Improve  before  the  nerve  swelling  reached  its  greatest  height.  The 
left  eye  remained  without  perception  of  light  for  Hfty  days  yet  at  no  time 
was  there  any  swelling  of  the  disc  or  congestion  of  the  retinal  veins. 
Both  nerves  subsequently  became  white,  although  vision  gradually  im- 
proved. Figure  148  shows  the  concentric  contraction  of  the  visual  fields 
that  existed  nine  months  after  operation. 

In  the  abo^e  case  blindness  of  both  eyes  preceded  visible  nerve 
changes,  differing  from  papilledema,  which  cioes  not  cause  blindness  and 
in  which  swelling  of  the  nerve  antedates  whatever  visual  disturbance 
occurs,  The  nature  oi  the  pathologic  process  can  only  be  inferred,  although 
the  development  of  choked  disc  in  the  right  eye  and  descending  atrophy 
in  the  left  indicate  a  retrobulbar  focus  of  inflammation  in  each  nerve, 
wh'xch  obstructed  the  lymph  current  in  one  (right)  but  not  in  the  other. 
There  is  little  doubt  that  the  conditions  were  produced  by  extension  of 
cerebral  meningitis  to  the  sheaths  of  the  nerves. 

In  postoperative  neuritis  the  picture  presented  by  the  swollen  discs 
is  not  always  the  same.  The  great  majority  look  like  cases  of  simple 
obstructive  edema.  Occasionally,  however,  the  disc  presents  the  appear- 
ance of  plastic,  exuclative  inflammation  (iitrabulbar  neuritis).  The  fol- 
lowing is  a  type  of  such  cases. 

Stereogkam  Gf).  Optic  Neuritis  in  Postoperative  Cerebral 
Sinus  Thrombosis.*  Woman  22  years  of  age.  Sinus  thrombosis  and 
meningitis  followed  operation  on  the  right  mastoid.  Seven  days  after 
operation  paresis  of  the  right  abducens  nerve  developed  and  an  ophthal- 
moscopic examination  revealed  swelling  ot  both  optic  nerves.  The  height 
of  the  discs  at  no  time  exceeded  1  mm.  The  swollen  tissues  of  the  disc 
had  a  reddish,  congested  cast  and  around  the  nerve  were  patches  of 
very  dense,  white  exudate.    The  veins  were  moderately  congested. 

After  the  neuritis  subsided  the  nerves  became  very  white,  presenting 
a    strongly   marked   picture   of    postneuritic    atrophv. 

This  case  differed  from  simple  edema  of  the  disc  in  the  following 
respects:  the  disc  was  but  moderately  swollen  and  the  neuritis  continued 
only  three  weeks,  yet  postneuritic  atrophy  resulted  with  vision  of  20/40 
and  contracted  fields    (Fig.    149). 

In  the  great  majority  of  postoperative  cases  swelling  of  the  nerve 
is  due  to  simple  edema  and  the  prognosis  as  to  vision  is  good.     In  the 


*  From   the  ear   service   of   Dr.   J.   F.    AIcKernon,    Manhattan    Eye,   Ear   and   Throat 
Hospital. 


>Mf,J^Jp-^C"  ^  > -^^^r  /-f-f^-f-t^ 


7\\ri»|L.\u«  vw.  T>(rttt|'U>uiXvtft  AtuNA^ 


Fig.  148 


'"10.  149. 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  231 

rare  cases  presumably  due  to  inflammation  situated  In  the  nerve  or  nerve 
sheaths,  vision  may  be  impaired  but  does  not  appear  to  be  permanently 
destroyed. 

Bibliography. 

Bartels,    Xeurol.    Ccntralb.    1908,    649;     Zeitschr.    f.    Aug.    XXI.    Heft.    1.    A.    f.    Aug. 

LVI,  267. 
Richards,    Personal   communication 
'      Rnttin,  The  Laryngoscope.  XXI.  Nov.   1911.  p.   1051. 
Ulhoff,  Graefe-Saemisch  Handh.   II,  Aufl.,  XI,  703. 

Optic  Atrophy.  Ophthalmoscopically,  atrophy  of  the  optic  nerve 
is  separated  into  three  principal  types,  i.  e.,  simple  atrophy  in  which  the 
nerve  fibers  disappear  by  a  purely  degenerative  process;  neiiritic  or  post 
inflammatory  atrophy,  in  which  the  nerve  fibers  are  destroyed  as  the 
result  of  Inflammation  or  congestion  involving  the  optic  disc,  and  retinitic 
atrophy,  in  which  destruction  of  the  nerve  fibers  is  secondary  to  retinitis. 

Simple  Optic  Atrophy.  In  simple  atrophy  destruction  of  the  nerve 
fibers  may  be  due  to  pressure,  injury,  remote  foci  of  disease  or  to  a 
general  disease  of  the  central  nervous  system.  The  best  example  of 
simple  atrophy  is  found  in  those  cases  where  the  nerve  has  been  severed 
or  destroyed  far  behind  the  eye  (Stergm.  25).  In  certain  grave  diseases 
of  the  nervous  system,  particularly  tabes  and  multiple  sclerosis,  optic 
atrophy  may  precede  by  years  development  of  other  symptoms  of  the 
disease.  Inasmuch  as  these  cases  are  seen  early  by  the  ophthalmologist, 
it  behooves  him  to  be  familiar  with  the  prominent  symptoms  of  the  under- 
lying affection. 

Tabls.  Simple  optic  atrophy  (grey  atrophy)  most  frequently  is 
due  to  tabes  and  is  the  first  prominent  manifestation  of  that  disease  in, 
approximately,  20  per  cent  of  all  cases.  Both  eyes  always  are  affected, 
but  in  one  atrophy  may  be  delayed.  An  optic  atrophy  is  recognized  as 
tabetic  by  two  symptoms  frequently  present  in  the  early  stage;  these  are, 
( 1 )  the  /\rgyl-Robertson  pupillary  reaction  in  which  the  pupils  remain  im- 
mobile on  exposure  to  light  but  contract  in  the  acts  of  convergence  and 
accommodation;  (2)  loss  of  patellar  tendon  reflex.  The  Argyl-Robertson 
pupil  is  diagnostic  of  cerebral  syphilis,  usually  tabes  or  paresis. 

Other  ocular  symptoms,  such  as  transitory  paralysis  of  an  extra- 
ocular muscle  and,  less  frequently,  contraction  or  dilation  of  the  pupils,  occur 
in   connection    with    tabes.  *      The    visual    fields    of   tabetic    atrophy    are 

*\  peculiarity  of  ocular  palsies  in  tabes  is  that  one  muscle  of  a  group  may  be  affected. 
also  that  the  paralysis  may  be  associated  with  loss  of  pupillary  light  reflex.  Permanent 
paralysis  of  a  single  ocular  muscle  usually  is   syphilitic. 


232  THE    FUNDUS    OCULI. 

concentrically  contracted  (Figs.  150-151).  Central  scotoma  has  hitherto 
been  regarded  as  rare  in  tabes  but  Fuchs  thinks  it  is  not  infrequent.  It 
is  yet  a  question  whether  such  cases  are  uncomplicated  tabes.  Contraction 
of  the  color  fields  keeps  pace  with  the  field  for  white  and  they  disappear 
in  physiologic  sequence,  first  green,  next  red  and  lastly  blue.  It  is  believed 
that,  when  tabes  commences  with  optic  atrophy,  development  of  inco- 
ordination and  other  symptoms  of  the  disease  Is  delayed.  Certainly, 
in  our  clinics  it  is  common  for  patients  with  tabetic  atrophy  to  remain  under 
observation  for  vears  without  developing  further  symptoms  of  tabes.  It 
appears,  however,  that  the  natural  course  of  tabes  is  extremely  slow  and 
ten  or  even  twenty  years  may  intervene  between  optic  atrophy  and  full  de- 
velopment of  the  disease.  Prognosis  as  to  the  ultimate  result  in  tabes 
is  extremely  unfavorable,  but  great  variations  exist  in  the  rate  of  progress. 
Deyl  gives  the  following  prognostics :  The  progress  will  be  slow  when  the 
nerve  is  very  white  and  vision  remarkably  good,  also  when  the  fields  for 
white  and  colors  contract  uniformly.  On  the  other  hand,  progress  will 
be  rapid  when  the  white  fields  remain  nearly  stationary  while  the  color 
fields  rapidly  contract,  and  especially  if  green  is  lost.  Tabes  occurs  in 
middle  life.     It  is  far  more  common  in  men  than  in  women. 

Probably,  atrophy  of  the  nerve  fibers  in  tabes  is  secondary  to  de- 
generation of  the  ganglion  cells  in  the  retina  although  this  point  has  not 
been  definitely  decided.  The  pathologic  process  appears  to  be  purely 
degenerative.  The  nerve  fibers  lose  their  myelin  sheaths,  become  varicose 
and  break  up,  differing  in  the  latter  respect  from  multiple  sclerosis,  in 
which  disease  the  continuity  of  nerve  fibers  is  not  destroyed.  In  the  late 
stage  of  tabetic  atrophy  the  nerve  consists  principally  of  neuroglia  and 
fibrous  connective  tissue    (Fig.    152). 

It  is  stated  that  at  least  95  per  cent  of  tabetics  have  had  syphilis,  and 
tabes  is  generally  regarded  as  a  late  manifestation  of  that  disease. 

In  searching  for  optic  atrophy  with  the  ophthalmoscope,  the  color  of 
the  disc  should  be  studied  with  a  dim  light  inasmuch  as  a  delicate  red  tint 
may  exist  which  a  strong  light  would  penetrate  and  cause  the  nerve  to 
appear  white.  In  simple  optic  atrophy  the  disc  at  first  appears  pale,  espe- 
cially on  the  temporal  side;  the  pallor  slowly  extends  to  the  nasal  side  and 
in  course  of  time  the  entire  disc  becomes  greyish  white.  The  disc  outline 
becomes  sharply  defined  and  the  adjacent  fundus. shows  no  disturbance. 
The  disc  may  be  depressed  but  the  edges  are  not  undermined;  the  physio- 
logic markings  of  the  lamina  cribrosa  become  very  distinct;  the  color  of  the 
disc  changes  to  a  grey  or  bluish  white  and  the  small  vessels  on  the  disc 
disappear  but  the  retinal  vessels  are  not  narrowed,  at  least  not  until  atrophy 


R. 


-y 


ToUtX*.  c/   0  {i  tic-  -A^"^  "i*^ 


Fie.  150. 


I     I.         i      ■  V     \     ,      , 


H  \\ 


Fi<'.  151. 


OPTIC    NEURITIS    AND    OP  IK'    AIKOPIii.  233 

Is  far  advanced.  If  the  retinal  arteries  are  diminished  in  size,  atrophy  of 
the  nerve  is  progressing  rapidly. 

Stereogram  67.  Simple  Optic  Atrophy  in  Tabes  (Grey  Atro- 
phy). Left  fundus  oculi  of  a  man  aged  51  years.  Both  eyes  affected. 
He  contracted  syphilis  twenty  years  ago,  for  which  he  underwent  pro- 
longed treatment  with  mercury.  Vision  has  been  noticeably  failing  for 
the  past  five  years  and  now  is  20/100;  there  is  poncentric  contraction  of 
the  visual  fields  (Fig.  150)  ;  the  pupils  do  not  react  to  light  but  contract 
when  the  patient  attempts  to  read  ( Argyl-Robertson  reaction)  ;  patellar 
tendon  reHex  abolished;  no  ataxia.  Ophthalmoscopic  Picture:  The  optic 
discs  are  bluish  white  in  color,  regular  in  outline,  sharply  defined  and 
slightly  depressed.  The  markings  of  the  lamina  cribrosa  are  very  distinct. 
There  is  no  disturbance  of  the  circumpapillary  pigment.  The  retinal 
vessels  are  normal   in  size  and  appearance. 

Diagnosis.  This  disc  presents  a  typical  picture  of  simple  atrophy. 
Postneuritic  atrophy  (Stergm.  69)  differs,  ophthalmoscopically,  in  that 
the  disc  is  a  dead  white,  the  markings  of  the  lamina  cribrosa  obscured,  the 
outline  of  the  disc  irregular  and  the  retinal  vessels  narrowed.  In  retinitic 
optic  atrophv  (Stergms.  22-44-53)  as  distinguished  from  simple  atrophy, 
the  disc  has  a  dirty  yellowish  color,  the  cribriform  markings  are  obscured, 
the  retinal  \essels  are  greatly  narrowed  and  the  retina  is  degenerated. 
The  grey  atrophic  nerve  of  glaucoma  is  recognized  by  the  undermined 
edges  of  the  disc. 

While  simple  optic  atrophy  is  most  frequently  due  to  tabes,  it  occurs 
also  in  several  other  diseases  of  the  nervous  system  of  which  the  following 
are  the  most  important. 

Ml  LEIPLE  Sclerosis.  In  multiple  sclerosis  the  brain  and  cord  con- 
tain widely  distributed  plaques  of  degeneration.  The  optic  nerve,  which 
is  a  prolongation  of  the  brain,  is  affected  in  about  one  half  of  all  cases. 
Morphologically,  the  nerve  fibers  lose  their  myelin  sheaths,  but  the  axis 
cylinders,  though  naked,  do  not  break  up  as  in  tabes,  neither  is  their  con- 
ductivity irrevocably  destroyed.  The  lesions  are  irregularly  distributed  and 
any  of  the  optic  nerve  fibers  may  be  Involved;  sometimes  only  the  axial 
bundle  is  affected.  In  opticus  cases  of  multiple  sclerosis  careful  examination 
usually  demonstrates  disease  of  both  nerves.  The  degeneration,  however, 
affects  the  two  nerves  so  unequally  that  the  atrophy  Is  clinically  regarded 
as  unilateral  In  about  75  per  cent  of  the  cases.  This  Is  in  marked  contrast 
to  tabecic  atrophy  which  always  is  bilateral,  the  two  nerves  being  affected 
in  about  equal  degrees.  Exceptionally,  neuritis  precedes  atrophy,  but  the 
majority  of  cases  are  atrophic  from  the  first.     The  ophthalmoscopic  pic- 


234  THE    FUNDUS    OCULI. 

ture  in  multiple  sclerosis  accords  with  the  anatomic  distribution  of  the 
plaques.  Thus,  the  nerve  may  show  atrophy  only  on  the  temporal  side  or  a- 
diffuse,  incomplete  blanching  may  exist,  but  the  blue,  sunken,  dead  disc 
of  -tabes  is  seldom  seen.  The  visual  fields  are  inconstant.  Minute,  mul- 
tiple central  scotomata  for  red  and  white  is  a  frequent  early  symptom 
(Fig.  H3).  Later,  extremely  irregular  peripheral  narrowing  occurs. 
Annular  scotoma  may  develop.  In  some  cases  scotomata  may  develop 
without  visible  changes  in  the  nerve.  Frequently,  the  extrinsic  eye  muscles 
are  paralyzed,  the  paralysis  being  incomplete  and  subject  to  wide  fluctua- 
tions. The  abducens  frequently  is  affected,  also  the  third  but  not  in  all 
its  branches.*  Paralysis  of  convergence  and  of  lateral  conjugate  move- 
ments is  common.  A  svmptom  of  importance  is  nystagmus  which  appears 
when  the  eyes  are  fixed  on  an  object,  especially  at  one  side.  The  pupils 
may  be  sluggish  but  never  are  affected  with  myosis  or  with  the  Argyl- 
Robertson  reaction  as  in  tabes.  The  optic  nerve  may  be  attacked  years 
before  other  symptoms  of  multiple  sclerosis  are  sufficiently  pronounced 
to  attract  attention. 

The  disease  usually  appears  between  the  fifteenth  and  thirtieth  years. 
It  is  verv  rare  after  thirty-five.  It  is  common  in  women  in  whom  the  fugi- 
tive, atypical  early  symptoms  frequently  are  ascribed  to  hysteria.  In  both 
multiple  sclerosis  and  hysteria  uncontrollable,  causeless  laughter  is  common. 
Tremor  accompanies  all  voluntary  movements  (intention  tremor).  Other 
general  symptoms  are  weakness  and  stiffness  in  the  legs,  vertigo,  apoplecti- 
form attacks,  headache  and  disturbance  of  speech.  Sometimes  the  intelli- 
gence suffers.  Multiple  sclerosis  does  not  lead  to  complete  blindness,  and 
it  is  characteristic  in  this  disease  for  the  ocular  symptoms  to  subside  and 
temporary  recovery  ensue.  Temporal  bleaching  of  the  nerve  should  always 
suggest  multiple  sclerosis,  although  it  occurs  also  in  other  conditions 
such  as  toxic  amblyopia  and  cerebral  syphilis. 

Stereogram  68.  Simple  Optic  Atrophy  in  Multiple  Sclerosis. t 
Right  eveground  of  a  woman  30  years  of  age  with  unilateral  optic  atrophy. 
For  the  past  four  years  she  has  been  affected  with  multiple  sclerosis. 
Cannot  walk  well;  has  dif^culty  with  speech;  memory  is  very  poor;  volun- 
tary motions  excite  tremor;  has  had  transient  paralysis  of  the  external 
ocular  muscles;  nystagmus  develops  when  she  attempts  to  fix  a  close  object. 
Right  eye,  vision  is  20/70:  irregular,  concentric  contraction  of  the  visual 
field.     I. eft  eye,  vision  20/30:     Visual  field  shows  small,  scattered  central 


*  Most    deplorable    results    follow    muscle   cutting    in    unreco,s;nized    multip'e    sclerosis 
and  tabes 

fPatient  was  seen  by  courtesy  of  Dr.  Edwin  G.  Zabriskie. 


Fig.  lo-J. 

Fis.  152 — Optic  Atrophy  of  Tabes.  Late  Stage.  The  Disc  is  Cupped.  The  Xerve 
Tissue  has  Disappeared,  the  Xerve  consisting  principally  of  Xeuroglia  and  Connective 
Tissue. 


L.£. 


n.E. 


UuJUipLe    S'clercris. 


Fig.  153. 


Fig  154. 
Oxycephaly,   Tower   Skull,   Turmschadel. 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  235 

scotomata.  This  nerve  is  not  ophthalmoscopically  atrophic,  nevertheless, 
it  is  affected.  In  the  right  eye  the  temporal  side  of  the  disc  is  atrophic  and 
decidedly  grey  in  color  although  the  atrophy  is  not  sufficiently  advanced 
to  reveal  the  markings  of  the  lamina  cribrosa.  On  the  nasal  side  the  disc 
presents  a  normal  color.  The  outline  of  the  nerve  is  sharply  defined  and 
there  is  no  evidence  of  previous  disturbance  in  the  surrounding  fundus.  The 
retinal  vessels  are  normal  in  size  and  not  sclerosed. 

Diagnosis.  In  this  eye  the  ophthalmoscopic  diagnosis  of  simple 
atrophy  depends  upon  the  light  grey  color  and  sharp  outline  of  the  disc,  the 
healthy  surrounding  fundus  and  the  normal  retinal  vessels.  Limitation  of 
the  atrophy  to  one  eye,  and  especially  to  the  temporal  side  of  the  disc, 
strongly  suggests  multiple  sclerosis  as  the  underlying  cause. 

Paresis.  Paresis  is  a  degenerativ^e  disease  of  the  nervous  system 
attended  by  progressi\'e  loss  of  bodily  functions,  including  a  blunted  sense 
of  the  proprieties  and  mental  enfeeblement  terminating  in  profound  de- 
mentia. In  this  affection  ocular  phenomena  are  common,  especially  ir- 
regularity and  inequality  of  the  pupils,  and  less  frequently  the  Argyl-Rob- 
ertson  pupillary  reaction.  Optic  atrophv  is  frequent  in  uncomplicated 
paresis  and  in  cases  complicated  with  tabes.  It  may  appear  early  and 
occasionally   antedates  other   symptoms. 

In  swingoniydia  both  choked  discs  and  optic  atrophv  have  been 
reported,  presumably  caused  by  pressure  of  a  distended  third  ventricle. 

Adiposity  \^'ith  optic  atrophy  usually  indicates  a  tumor  of  the  pituitary 
body  which  influences  body  growth. 

Hercdilary  Optic  Atrophy  is  preceded  by  retrobulbar  neuritis,  but  the 
disc  presents  the  characteristics  of  simple  atrophv.  "Phis  peculiar  disease 
attacks  only  the  males  of  an  affected  family,  but  is  transmitted  through  fe- 
males who  themselves  escape  the  affection.  As  a  rule  the  disease  develops 
during  puberty  or  early  manhood. 

In  lateral  sclerosis  of  the  cord  optic  atrophy  may  appear  and  present 
the  same  symptoms  as  in  tabes. 

Optic  atrophy  may  be  associated  with  congenital  or  acquired  deform- 
ities of  the  cranium.  In  most  of  these  cases  the  optic  foramen  has  been 
found  to  be  very  small,  but  it  is  uncertain  whether  the  atrophy  is  always 
due  to  narrowing  of  the  foramen  or  both  conditions  proceed  from  a  common 
cause.  Figure  154  shows  a  girl  four  years  of  age  with  a  "  tower  skull." 
She  is  nearly  blind  from  progressive  optic  atrophv.  In  this  case  it  may  be 
assumed  that  the  exophthalmus  and  optic  atrophy  are  caused  by  pressure 
of  the  confined  brain  upon  the  bones  which  form  the  roof  of  the  orbit 
and  the   optic   foramen. 


236  THE    FUNDUS    OCULI. 

Extreme  optic  atrophy  follows  penetrating  wounds  of  the  orbit  which 
sever  the  nerve  or  cause  hemorrhage  and  compression.  Frequently  a  blow 
on  the  edge  of  the  orbit  causes  a  fracture  which,  extending  through  the 
optic  foramen,  produces  injury  and  atrophy  of  the  nerve  (Stergm.  25). 
In  these  cases  blindness  may  exist  for  weeks  before  the  nerve  becomes  white. 
The  cases  reported  of  optic  atrophy  in  young  adults  associated  with  per- 
sistent watery  discharge  from  the  nose,  probably  were  cases  of  hydro- 
cephdlus  intt'ruiis.  Simple  optic  atrophy  has  been  attributed  to  a  great 
variety  of  diseases,  but  it  is  probable  that  in  most  of  them  atrophy  was 
preceded  by  papillitis. 

Neukitic  Optic  Atrophy.  Atrophy  of  the  optic  nerve  which  has 
been  preceded  by  neuritis  or  papilledema  is  designateci  neuritic — or  post- 
neuritic atrophv,  in  contradistinction  to  simple  degeneration  of  the  nerve. 
A  knowledge  of  the  inflammatory  processes  which  lead  to  optic  atrophy 
is  essential.  The  optic  nerves,  both  in  structure  and  function,  are  a  continua- 
tion of  the  brain,  and  the  brain  membranes  (dura,  arachnoid  and  pia) 
ensheath  them  from  the  cranial  ca\'ity  to  the  eye;  consequently,  diseases 
of  the  brain  substance  may  invade  the  nerves  by  direct  extension,  or 
meningeal  inflammations  may  spread  to  the  nerve  sheaths.  The  optic 
nerves  may  be  the  seat  also  of  infectious  metastases.  Basilar  extrava- 
sations of  blood  sometimes  flow  into  the  optic  canals  and  form  a  hematoma 
of  the  sheath.  Inflammation  of  the  sheath  is  termed  per'uiearitis.  In  this 
condition  the  intervaginal  space  is  filled  with  exudates  and  the  inflammatory 
processes  usually  extend  to  the  optic  nerve.  Perineuritis  may  be  due  to 
syphilis,  tuberculosis,  meningitis,  injuries,  periostitis  of  the  orbit,  orbital 
abscess,  etc.  Inasmuch  as  the  intervaginal  spaces  of  the  optic  nerve  com- 
municate directly  with  the  subdural  and  subarachnoid  spaces  of  the  brain, 
it  follows  that  heightened  intracranial  pressure  leads  to  distension  of  the 
nerve  sheaths  and  edema  of  the  nerv-e  head  fFig.  139). 

Parenchymatous  inflammation  of  the  optic  nerve  frequently  is  due 
to  syphilis.  Gummatous  infiltration  of  the  optic  tract  is  most  frequent  at 
the  chiasm,  but  any  portion  of  the  nerve  may  be  attacked.  The  histologic 
changes  consist  of  cellular  infiltration  and  formation  of  granulation  tissue, 
which  is  replaced  by  a  cicatrix.  In  other  cases  the  tissues  undergo  necrosis, 
but   in  either  event  the  nerve  fibers   are   destroyed   and   atrophy   results. 

Severe  or  prolonged  swelling  of  the  intraocular  end  of  the  nerve,  of 
either  inflammatory  or  noninflammatory  origin,  is  followed  by  atrophy, 
but  tissue  disturbance  is  greater  and  atrophy  occurs  earlier  in  the  inflam- 
matory type.  In  postneuritic  atrophy  the  nerve  fibers  perish  while  neuroglia 
and  newly  formed  connective  tissue  cover  the  disc  and  enwrap  the  vessels, 


OPTIC    XEUKIIIS    AND    OPTIC    ATROPHY.  237 

producing  an  ophthalmoscopic  picture  quite  different  from  that  of  simple 
atrophy.  In  neuritis  and  papilledema,  infiltration  of  the  tissues  extends 
for  a  short  distance  beyond  the  n^rve  Into  the  adjacent  retina  and  choroid. 
Evidence  of  this  remains  after  the  subsidence  of  swelling,  in  the  form  of  clr- 
cumpapillary  atrophy  and  pigment  disturbance.  I'he  neuroglia  on  the  disc 
loses  its  transparency  and  this.  In  connection  with  newly  formed  connective 
tissue,  may  hide  the  markings  of  the  lamina  and  fill  all  depressions  on  the 
disc.  Neuritic  atrophy  follows  a  state  of  either  passive  or  active  venous  en- 
gorgement, and  the  veins  remain  tortuous  long  after  the  swelling  has 
subsided  and  the  disc  become  white.  (Gradually,  however,  contraction  of 
the  new  tissue  in  the  nerve  constricts  and  narrows  the  retinal  vessels  which 
now  may  exhibit  degenerative  changes.  In  the  early  stage  of  neuritic 
atrophy  the  nerve  may  retain  a  reddish  grey  color  but,  when  the  scar  tissue 
is  organized  and  the  capillary  circulation  on  the  disc  abolished,  the  surface 
becomes  dead-white  and  the  outlines  sharply  defined.  The  disc  also  may 
appear  smaller  than  normal  and  irregular  in  outline.  All  the  manifestations 
described  are  more  marked  after  inflammatory  neuritis  than  after 
papilledema. 

The  prognosis  depends  upon  the  amount  of  destruction  wrought  by 
preceding  Inflammation  and  by  contraction  of  cicatricial  tissue  and,  as  a 
rule,  is  not  favorable.  Nevertheless,  It  is  better  than  in  tabetic  atrophy, 
which  is  progressive,  while  neuritic  atrophy,  once  established.  Is  stationary 
unless  due  to  syphilis,  which  may  relapse  at  any  time. 

Stereogram  69.  Neuritic  Optic  Atrophy.  Right  eyeground  of 
a  woman  29  years  of  age.  Nine  years  ago  she  acquired  chancre  on  the  lip 
from  her  husband  and  was  treated  at  the  hospital  for  over  a  vear.  Four 
years  later  she  was  ill  for  a  long  time  and  suffered  from  violent  headaches, 
vertigo  and  trouble  with  vision.  Ihe  Wassermann  reaction  for  syphilis  is 
positive.  Central  vision  Is  20/50  in  each  eye.  There  is  concentric  con- 
traction of  the  visual  fields.  The  ophthalmoscopic  picture  is  that  of 
postneuritic  optic  atrophy.  The  white  optic  disc  Is  rendered  very  con- 
spicuous by  contrast  with  the  surrounding  fundus.  Its  outlines  are  sharp 
but  slightly  irregular.  The  circumpapillary  pigment  Is  superabundant  and 
in  some  places  appears  to  have  been  pushed  away  from  the  nerve.  The 
disc  presents  a  smooth,  dense  surface  on  which  all  physiologic  depressions 
and  markings  are  obscured.  All  the  retinal  vessels  are  uniformly  con- 
tracted and  the  smaller  branches  have  disappeared.  Some  of  the  arteries 
have  sclerosed  walls,  as  shown  by  white  bordered  blood  columns. 

Diagnosis.  Neuritic  atrophy  is  ophthalmoscopically  distinguished  by 
a  very  white,  smooth  surface  of  the  nerve  as  compared  with  the  grey, 


238 


THE    FUNDUS    OCULI. 


cribriform  disc  of  tabes  or  the  yellowish  disc  of  retinitic  atrophy.  In  tabetic 
atrophy  the  retinal  vessels  are  normal  In  retinitic  atrophy  the  vascular  de- 
genei*ation  is  greater  than  in  neuritic  atrophy. 

The  following  table  applies  only  to  typical  cases  of  optic  atrophy: 


SIMPLE 
ATROPHY. 

NEURITIC 
ATROPHY. 

RETINITIC 
ATROPHY. 

GLAUCOMA. 

Color    of    Disc. 

Grey  or  bluish- 
white. 

Dead   white. 

Dirty     yellowish 
white. 

Greenish  grey. 
Usually  darker 
than  simple  atro- 
phy. Deep  periph- 
eral  shadow. 

Outluie. 

Sharp,      regular, 
.lormal    size. 

Early     stage 
blurred  ;    later, 
sharp    but    irregu- 
lar ;      may    appear 
small. 

Indistinct.     Nor- 
mal  size. 

Sharp.  May  ap- 
pear enlarged. 

1  a  til  ilia 
Cribrcsa. 

P  li  y  s  i  o  1  o  g  i  c 
markings  very  dis- 
tinct. 

Usually,      mark- 
ings  are  obscured. 

Markings      o  b  - 
scured. 

Markings  very 
distinct. 

r.urface   of 
Disc. 

Normal      or 
slightly    depressed. 

May  be   covered 
with  opaque  tissue. 

Opaque  tissue 
usually    less    abun- 
dant than  in  neuri- 
tic  atrophy. 

E  xc  a  vated  to 
edge  of  disc.  Ex- 
c  a  V  a  t  i  o  n  com- 
mences o  n  tem- 
poral  side. 

C-rniiiifat>il- 
lary    Region. 

Unaltered. 

Irregularly  atro- 
phic.   Pigment   line 
shoved  away  from 
margin. 

Extensive   chori- 
oretinal   changes 
usually  present. 

Light  colored, 
depigmented  ring, 
the  glaucomatous 
halo. 

Ivttinal 
Vessels. 

Normal.      M  a  y 
b  e     narrowed     i  n 
very  old  cases. 

Ea'-ly     stage, 
veins  are  tortuous ; 
later,    a  1 1    vessels 
narrowed. 

Vessels     nar- 
rowed  and   degen- 
erated. 

Veins  dispropor- 
tionately filled  ; 
arteries  narrowed ; 
angioscle- 
rosis ;  vessels 
pushed  to  nasal 
side;  arterial  pul- 
sation if  tension  is 
very  high. 

I  isual    Fields. 

In  tabes  concen- 
tric contraction. 

Irregular  or  con- 
centric contraction. 

Depends     on 
for  m    of    fundus 
lesion. 

Peripheral  con- 
traction ;  begins  in 
nasal  field ;  also, 
sector  defects  con- 
nected with  blind 
spot. 

Course. 

Progressive. 

Nonprogressive. 

Nonprogressive 
unless  fundus  dis- 
ease  recurs. 

Progressive. 

OPTIC    NEURITIS    AND    OPTIC"    AIROPHY.  239 

Compression  of  the  Optic  Nerve  in  Arteriosclerosis.  In  cere- 
bral arteriosclerosis,  the  mechanical  pressure  of  diseased  arteries  upon  the 
optic  nerve  may  produce  either  optic  atrophy  or  neuritis. 

Liebrecht  designates  three  points  in  the  course  of  the  nerve  particularly 
subject  to  arterial  pressure.     These  are: 

( 1 )  At  the  inner  opening  of  the  optic  canal.  This  canal,  which  trans- 
mits the  optic  nerve,  consists  of  a  bony  portion,  4  mm.  in  length,  and  a 
fibrous  portion  which  extends  into  the  cranial  cavity  for  a  distance  of  2  mm. 
The  floor  of  the  fibrous  extension  is  formed  in  part  by  the  carotid  artery, 
while  the  roof  is  covered  with  a  fold  of  dura,  the  free  edge  of  which  is 
tense  and  sharp.  Between  the  carotid  and  roof  lies  the  optic  nerve  which 
here  is  subjected  to  the  vigorous  pulsations  of  the  artery.  Under  normal 
conditions  this  creates  no  disturbance,  but  when  the  carotid  is  greatly 
dilated  and  sclerosed,  the  nerve  will  be  crushed  against  the  free,  sharp 
edge  of  the  roof. 

(2)  The  ophthalmic  artery  leaves  the  cranial  cavity  through  the  optic 
canal,  where  it  lies  below  and  to  the  outer  side  of  the  optic  nerve.  Marked 
distension  and  hardening  of  the  artery  within  the  narrow,  unyielding  walls 
of  this  canal  always  result  in  compression  and  deformity  of  the  nerve. 
According  to  Liebrecht  it  is  only  in  the  fibrous  portion  of  the  canal  that  the 
nerve  can  be  injured  by  pressure  of  the  artery;  because,  after  the  artery 
enters  the  bony  canal,  it  becomes  invested  with  a  dense  dural  sheath  which 
completely  isolates  it  from  the  nerve. 

(3)  Midway  between  the  inner  opening  of  the  optic  canal  and  the 
optic  chiasm  the  nerve  passes  between  the  carotid  and  anterior  cerebral 
arteries.  Should  these  two  vessels  become  sclerosed  they  will  exert  undue 
compression  on  the  nerve. 

In  addition,  sclerosis  and  aneurismal  dilation  of  the  carotids  or  the 
arteries  in  the  circle  of  Willis,  may  destroy  more  or  less  of  the  optic  nerves 
or  chiasm,  thereby  producing  some  of  the  various  forms  of  hemianopsia. 

Of  the  above  sites  of  arterial  pressure  upon  the  optic  nerve  the 
most  frequent  is  within  the  optic  canal.  Here,  the  pulsations  of  a  sclerosed 
ophthalmic  artery  will  produce  a  deep  longitudinal  sulcus  upon  the  lower 
surface  of  the  nerve,  creating  the  condition  known  as  "  middle  furrow 
formation."  In  case  the  artery  is  markedly  dilated  and  sclerosed,  the 
nerve  may  thus  be  split  into  two  lateral  parts  connected  above  only  by  a 
thin  bridge  of  tissue.  The  frequent  discovery  at  autopsies  of  these  furrows 
in  the  nerve  led  to  the  assumption  that  the  papillo-macular  bundle  of  fibers 
must  often  be  destroyed  in  this  manner  and  thus  produce  absolute  central 


240  THE    FUNDUS    OCULI. 

scotoma.  In  those  cases,  however,  where  deep  furrows  or  even  splitting 
of  the  nerve  have  been  found  post-mortem,  a  history  of  scotomata  during 
life  was  lacking.  Nevertheless,  in  most  of  the  cases  examined  by  Otto 
and  by  Liebrecht,  in  which  the  nerve  had  been  subjected  to  extreme  pressure, 
microscopic  examination  showed  actual  atrophy  of  some  nerve  fiber  bundles. 
These  findings,  if  accurate,  lead  to  the  inevitable  conclusion  that  the  atro- 
phied nerve  fibers  must  have  been  represented  in  the  retina  by  blind  areas, 
even  though  functional  disturbance  was  not  recognized  by  the  patient.  On 
examining  the  visual  fields  of  80  senile  subjects  for  symptoms  of  pressure 
atrophy,  Liebrecht  found  two  cases  of  absolute  central  scotoma  and  two 
of  paracentral  scotoma.  Others  exhibited  marked  contraction  of  the  fields 
which  did  not,  however,  prevent  useful  vision.  These  defects  were  at- 
tributed to  the  mechanical  action  of  sclerotic  vessels  upon  the  optic  nerve, 
toxic  amblyopia  having  been  excluded.  C.  S.  Bull  had  under  observation 
for  years  cases  with  scotomata  and  progressive  loss  of  vision  which  he 
attributed  to  nerve  atrophy  from  pressure  of  sclerosed  vessels.  Further- 
more, he  was  strongly  disposed  to  regard  so-called  simple  glaucoma  as  a 
descending  nerve  atrophy  due  to  the  same  cause. 

If  otherwise  unaccountable  defects  exist  in  the  visual  fields  of  a  patient 
with  vascular  degeneration,  especially  if  the  nerve  head  is  white,  a  diagnosis 
of  arteriosclerotic  pressure  atrophy  may  be  warranted.  A  white  disc, 
however,  is  not  an  essential  feature  of  the  symptom  complex,  inasmuch 
as  functional  disturbance  long  precedes  bleaching  of  -the  disc.  This  is 
due  to  the  fact  that  the  point  of  pressure  on  the  nerve  is  situated  far  behind 
the  place  of  entrance  for  the  retinal  vessels  where  it  does  not  interfere 
with  the  circulation  and  nutrition  of  the  disc.  Consequently,  there  will 
be  no  bleaching  of  the  nerve  until  the  defunct  fibers  have  been  replaced 
by  connective  tissue.  In  these  cases  other  symptoms  of  cerebral  arterio- 
sclerosis may  be  present,  namely,  vertigo  on  sudden  movement  of  the  head 
or  eyes,  diffuse  headache,  irritable  disposition,  failure  of  memory,  hal- 
lucinations of  vision  and  even  dementia. 

The  most  meager  evidence  exists  that  complete  blindness  ever  is 
due  to  pressure  of  sclerotic  arteries  upon  the  optic  nerve.  These  so- 
called  nerves  are  prolongations  of  the  brain  and,  like  all  brain  tissue, 
will  endure  without  loss  of  function  an  enormous  degree  of  pressure  if 
gradually  applied.  It  is  very  doubtful  if  "  middle  furrows  "  in  the  optic 
nerve  cause  appreciable  visual  disturbance,  unless  due  to  extreme  and 
progressive  pressure  or  pressure  suddenly  exerted.  In  the  few  cases  of 
optic  atrophy  recognized  during  life  and  demonstrably   due   to  pressure 


OPTIC    NEURITIS    AND    OPTIC    ATROPHY.  241 

from  diseased  arteries,  the  anatomical  changes  were  of  the  coarsest  char- 
acter. Thus,  Byron  Bramwell  described  a  case  of  temporal  hemianopsia 
with  pale  discs  which  terminated  in  blindness  and  mania.  Autopsy  dis- 
covered an  enormous  aneurism  pressing  on  the  optic  nerves,  chiasm  and 
tracts. 

Rampoldi  reported  the  case  of  a  man  affected  with  headache,  poor 
memory,  optic  atrophy  and  very  low  vision.  Autopsy  showed  diffuse 
arteriosclerosis  and  a  ruptured  aneurism  of  the  left  internal  carotid. 

Pechin-Rollln  observed  a  case  with  optic  atrophy  and  oculo-motor 
paralysis  of  the  right  eye  and,  in  the  left  eye,  ptosis  with  paralysis  of 
the  superior  rectus.  Post-mortem  examination  revealed  extreme  sclerosis 
of  the  carotid  and  ophthalmic  arteries.  The  paralysis  was  attributed  to 
pressure  of  the  sclerosed  carotid  upon  the  nerves  in  the  cavernous  sinus. 

The  histologic  changes  produced  in  the  optic  nerve  by  extreme  pres- 
sure of  the  ophthalmic  artery  in  the  optic  canal  are  regarded  by  Bernheimer, 
Otto  and  Liebrecht  as  those  of  non-inflammatory  pressure  atrophv.  Cer- 
tain nerve  fibers  lying  at  the  bottom  of  the  pressure  furrows  lose  their 
medullary  sheaths  and  disappear.  From  this  point  an  ascending  and  de- 
scending atrophy  of  the  affected  fibers  rapidly  ensues.  In  course  of  time 
new  blood  vessels  may  develop  and  be  followed  by  formation  of  connective 
tissue.  From  the  microscopic  examination  of  similar  cases,  Oppenheim  and 
Siemerling  conclude  that  a  retrobulbar  neuritis  is  excited  at  the  point  of 
pressure.  They  found  all  the  evidence  of  chronic  Inflammation,  i.  e.,  cel- 
lular proliferation.  Increased  vascularity  and  new- formed  connective  tissue. 
All  microscopic  reports  which  describe  atrophic  nerve  fibers  and  Interstitial 
sclerosis  situated  in  the  periphery  of  the  optic  nerve  should  be  regarded 
with  suspicion,  inasmuch  as  the  once  accepted  "  peripheral  atrophy  "  Is 
now  known  to  be  the  normal  formation  which  exists  In  all  optic  nerves, 
even  In  the  new-born. 

Instead  of  resulting  In  atrophv,  pressure  of  sclerotic  arteries  on  the 
nerve  mav  cause  choked  disc.  In  1887  Michel  reported  a  case  of  double 
choked  discs  with  retention  of  normal  vision  and  lull  visual  fields.  The 
cause  was  shown  to  be  distension,  tortuosity  and  hardening  of  both  internal 
carotids  which  obstructed  the  outflow  of  Ivmph  from  the  optic  canals, 
causing  edema  of  the  nerves  and  distension  of  their  sheaths.  Two  similar 
cases  were  reported  by  Stoltlng.  The  first  exhibited  choked  disc  in  one 
eye  and  a  dirty  red  nerve  head  in  the  other.  There  was  also  paresis  of 
both  abducens.  The  clinical  diagnosis  was  brain  tumor.  At  necropsy 
no   tumor  was   found,   but   there   was  extreme   sclerosis    of  the   cerebral 

16 


242  THE    FUNDUS    OCULI. 

arteries.  The  second  case  resembled  the  first  including  paralysis  of  the 
abducens,  but  no   anatomical  examination  was   secured. 

Stolting  calls  attention  to  the  following  features  presented  by  these 
cases  which  may  prove  to  be  characteristic  of  cerebral   arteriosclerosis: 

(  I )  The  long  duration  of  the  aftection,  which  was  ten  years  in  one 
case  and  six  years  in  the  other. 

(2)  The  optic  neuritis  showed  no  tendency  to  pass  into  atrophy, 
which  would  not  be  the  course  pursued  by  a  neuritis  dependent  on  any 
other  chronic  disease. 

(3)  Paralysis  of  the  abducens,  probably  due  to  pressure  by  a  scle- 
rosed carotid  on  the  sixth  nerve  within  the  cavernous  sinus. 

Among  the  above  symptoms,  oculo-motor  paralysis  is  the  most  char- 
acteristic of  cerebral  arteriosclerosis  and  has  been  observed  in  several 
authentic  cases.*  The  sixth  nerve  in  passing  through  the  cavernous  sinus 
lies  in  contact  with  the  internal  carotid  and  is  the  one  most  frequently 
aftected,  but  the  other  ocular  nerves  may  be  involved  if  the  artery  is 
greatly  distended.  In  a  case  reported  by  Ritter  there  was  complete  oph- 
thalmoplegia of  the  left  eye,  due  to  aneurismal  dilation  and  rupture  of 
the  ophthalmic  artery.  The  blood  clot  lay  partly  within  the  cranial  cavity, 
partly  in  the  apex  of  the  orbit. 

Optic  neuritis  due  to  arteriosclerosis  is  not  necessarily  of  Intra- 
cranial origin.  Sometimes  the  only  ocular  manifestation  of  arteriosclerosis 
is  neuritis  due  to  disease  of  the  vessels  distributed  to  the  head  of  the  nerve 
which  here,  as  in  the  retina,  results  in  edema,  exudation  and  hemorrhage. 

The  ophthalmoscopic  picture  of  albuminuric  retinitis  has  been  ob- 
served In  uncomplicated  arteriosclerosis.  In  such  cases  it  is  possible 
that  the  white  retinal  exudates  may  be  lymph  which  has  escaped  from 
the  perivascular  sheaths,  as  the  result  of  obstruction  within  the  cranial  cavity 
by  a  distended  Internal  carotid.  Purtscher  describes  a  case  as  lymphor- 
rhagla  in  the  fundus  In  which,  following  fracture  of  the  skull,  the  retina 
was  covered  with  white  exudates. 

Bibliography. 

Bernheimer,  A.  f.   O.,  XXXVIL  2,  S.  37;    3,  S.  36. 
Bramwell,  Byron,  Edinb.   Med.   tour.,  Apl.  1887,  p.  918. 
Bull,  C.  S.,  Annals  of  Oph.,  XIH,  1.  Jan.  1904. 
Frankel.  Virchow.  A.  f.  Path.  Anat.  79.  S.  509. 
Greeff,  Lehrb,  d.  Spec.   Path.  Anat.  Auge,  S.  416. 
Halby,   Inang.   Dissert.,  Kiel,  1902. 


*Pechin  et  RoIIin,  Halby,  Frankel,  Stolting,  Rigel,  Ritter,  Michel. 


oi'lJC    NEURITIS    AND    OPTIC    ATROPHY.  243 

Liebrecht,  A.  f.  A.,  XLIV,  1902,  193. 

Michel,   A.   f    ().,   XXIII,   1887,  2. 

Moses,  Inaug.  Dissert.   VViirtsherg,   1S9(). 

Oppenheim-Siemerling,  Chatite,  Annalen,  1897. 

Otto    Untersuch.  ii.   Schnervcn   Veranderungen  bei   Arterisklerosie,  Berlin,  1893 ;    A.   f . 

A.,  XLIII,  2. 
Pechin  et  Rollin.  A.  d'O.  XXlll,  576. 

Purtscher.  Bcricht  u.  d.  36  Versaml.  d.  Oph.  Gesel.   Heid.  1910,  294. 
Rampoldi.    |ahrs.   Bericht  d.   O.,   1892,   XT II,  327. 
Rigel,    Munich    Med.    Woch.,   1899,    1133. 

Rittcr,   Rcf.   by   Liel)recht.   Gesell.    f.    Natur   n.    Heilkunde,    Dresden,    1887. 
Scultet,  Zietert  a.  Dem.  Traite,  d.  Med.  d.  Yeux,  Paris,  1818. 
Stoking,  K.  M.  f.  A.,  XLIII,  2,  129. 
Von  Graefe,  Ref.  in  Wilbrand  u.  Saenger  (Angiecktasie,  1808,  33). 


Chaptkr  XII. 
MYOPIA. 

In  the  myopic  eye,  parallel  rays  of  light  come  to  a  focus  before  they 
reach  the  retina,  thus  producing  a  blurred  image.  In  the  v^ast  majority 
of  cases  this  is  due  to  elongation  of  the  posterior  half  of  the  eyeball  and 
is  termed  axial  myopia.  Other  exceptional  and  theoretical  causes  are: 
increased  curvature  of  the  cornea  or  lens,  known  as  curvature  myopia; 
an  abnormally  high  index  in  the  refracting  media,  called  index  myopic,  • 
and  forward  displacement  of  the  lens. 

Axial  Myopia.  Despite  the  enormous  amount  of  research  work 
expended  upon  myopia,  neither  its  etiology  nor  pathology  has  been  satis- 
factorily described. 

Etiology.  The  principal  reasons  which  have  been  advanced  to 
explain  myopic  elongation  of  the  globe  are: 

(1)  Subacute  inflammation  of  the  choroid  and  sclera  (sclerocho 
roiditis  posterior).  This  theory  is  no  longer  tenable,  the  microscope 
having  failed  to  show  evidence  of  primary  inflammation  except  in  cases 
of  syphilis  in  adults.  The  hyperemia,  exudation  and  cicatrization  found 
in  ordinary  myopia  are  reparative  processes  which  follow  stretching  and 
laceration  of  the  membranes.  Von  Graefe,  who  advanced  this  theory, 
subsequently  changed  his  views. 

(2)  Near  JJ^ork  which  requires  efforts  of  accommodation  and  c"  t- 
vergence,  was  suggested  first  by  Kepler  as  the  cause  of  myopia.  Probably 
this  is  causative  only  in  eyes  predisposed  to  the  disease,  but  it  is  highly 
injurious  in  developed  cases.  A  spurious  myopia  due  to  spasmodic  con- 
traction of  the  ciliary  muscle  may  occur  in  an  emmetropic  or  hyperopic 
eye.  Bonders  showed  that  near  work  could  not  be  the  only  cause  of 
myopia,  inasmuch  as  the  highest  grades  may  be  found  among  the  illiterate 
and  those  who  do  no  close  work. 

(3)  Compression  of  the  Eye  by  the  Extrinsic  Muscles.  When 
it  was  established  that  myopia  was  attended  by  distortion  of  the  globe,  it 
was  at  once  suspected  that  action  of  the  extrinsic  muscles  was  causative. 
although  opinions  differed  as  to  the  mechanism  of  their  action.  Stilling 
considered  that  the  two  obliques,  which  enwrap  the  eye,  were  responsible, 

244 


MYOPIA.  245 

while  Arlt  believed  that  muscular  pressure  on  the  vortex  veins  produced 
congestion  which  resulted  in  distension.  Other  writers  have  considered 
action  of  the  internal  and  external  recti  as  sufficient  to  elongate  the  eye- 
ball. Stilling  stated  that  when  the  roof  of  the  orbit  was  low  the  pulley 
of  the  superior  oblique  was  so  misplaced  that  this  muscle  exerted  undue 
pressure  upon  the  posterior  segment  of  the  eye,  and  his  measurements 
tended  to  prove  that  myopes  have  an  abnormally  low,  broad  orbit.  This 
theory  has  not  been  supported  by  the  anatomic  investigations  of  others. 
Muscular  compression  undoubtedly  injures  an  elongated  eye,  but  it  is 
improbable  that  it  could  change  the  form  of  a  normal  globe.  It  is  reason- 
able to  suppose,  however,  that  the  oblique  muscles  influence  the  form  of 
a  distensible  sclera  and  determine  the  position  of  a  posterior  staphyloma. 

(4)  Short  Oplic  Nerve.  According  to  this  theory  the  nerve  is 
too  short  and  pulls  upon  the  back  of  the  eye  when  the  posterior  pole  is 
rotated  outward  as  in  convergence  (Hasner,  Weiss).  Anatomic  investi- 
gations have  failed  to  establish  this  view. 

(5)  Congenital  Jl'ecik}iess  of  the  Selern  at  the  Posterior  Pole. 
Schnabel  says  that  congenital  anomalies  in  the  choroid  and  sclera  are  con- 
stant in  the  posterior  staphyloma  of  Scarpa. 

(6)  Co}ilr action  of  the  Ciliary  Muscle.  The  view  that  ciliary  spasm 
is  in  some  way  connected  with  the  development  of  low  myopia  persists, 
although  it  is  inconceivable  that  the  form  of  the  globe  can  be  influ- 
enced by  action  of  the  ciliary  muscle.  Spurious  myopia  due  to  con- 
traction of  the  ciliary  muscle  often  appears  to  precede  the  development 
of  nonprogressive  myopia,  but  it  is  not  clear  what  relationship  exists 
between  the  two  conditions,  although  it  is  conceivable  that  commencing  elon- 
gation of  the  globe  may  excite  ciliary  spasm. 

Little  is  kn.own  regarding  the  influence  of  race  upon  myopia.  Parsons 
states  that  myopia  is  very  common  among  the  educated  natives  of  India. 
Stephenson  among  918  boys  and  231  girls  in  a  London  school,  found 
the  percentage  of  myopia  in  Jewish  bovs  to  be  six  times  greater  than 
among  the  others,  and  among  the  Jewish  girls  it  was  three  and  a  half 
times  greater.     Myopia  is  more  common  among  women  than  among  men. 

Myopia  appears  to  be  hereditary  but  to  what  extent  has  not  been 
determined.  Pfliiger,  among  55  families  in  which  one  or  both  parents 
were  myopic,  found  that  71  per  cent  of  the  children  had  myopia. 
Schneller,  among  1439  scholars  of  the  higher  schools  found  that  only 
37  per  cent  of  the  myopes  had  myopic  parents.  Myopia  is  very  rare 
in  new-born  infants. 


246  THE    FUNDUS    OCULI. 

Mvopia  is  divided  into  two  classes  which  may  prove  to  be  distinct 
affections,  although  Schnabel  asserts  the  identity  of  all  forms  of  myopia. 
The  first  type  has  an  average  range  of  from  2  to  8  dioptres.  It  develops 
coincidently  with  school  life  and  becomes  stationary  about  the  18th  or 
20th  year.  This  is  the  myopia  of  adolescence,  known  also  as  nonpro- 
gressive— and  as  school  myopia.  The  seconci  type  is  known  as  pro- 
gressixe  or  pernicious  myopia  and  also,  from  its  serious  consequences,  as 
malignant  myopia. 

Stereogram  70.  Nonprogressive  Myopia.  Right  eyeground  of 
a  man  ageci  30  years.  Bookkeeper.  With  a  concave  lens  of  6.5  dioptres 
vision  is  20,'20.  His  refraction  has  not  changed  since  he  was  18  years 
of  age.  The  ophthalmoscopic  picture  is  characteristic  of  benign  myopia. 
The  retinal  pigment  is  sufficiently  rarefied  to  reveal  the  choroitial  ves- 
sels. The  vertical  retinal  vessels  are  slightly  inclined  toward  the  tempo- 
ral side  and  all  vessels  pursue  a  straight  course.  The  optic  disc  is  normal 
in  color.  On  the*temporal  side  it  is  bordered  by  a  white,  crescentic  figure 
the  outer  border  of  which  is  marked  by  a  line  of  pigment.  This  figure 
is  known  as  the  myopic  crescent  or  conus. 

Diagnosis.  Mvopia  is  diagnosticated  by  testing  the  refraction  rather 
than  by  the  ophthalmoscopic  appearance,  although  the  conus  and  straight 
retinal  vessels  in  this  fundus  are  characteristic. 

Bv  the  direct  method  of  ophthalmoscopic  examination  the  myopic 
fundus  is  poorly  illuminated  and  the  cietails  magnified.  If  the  myopia  is 
high,  a  direct  examination  is  difficult.  With  the  indirect  method,  how- 
ever, the* picture  becomes  very  brilliant  and  sharply  defined. 

Anatomic  Changes.  The  myopic  crescent  or  conus  shown  in  the 
preceding  stereogram  is  the  most  constant  ophthalmoscopic  feature  in 
myopia,  being  present  in  about  70  per  cent  of  all  cases.  Conus  must  not 
be  confused  with  posterior  bulging  of  the  sclera  (staphyloma  of  Scarpa), 
which  is  a  ciistinct  condition.  While  conus  occurs  in  all  degrees  of  myopia, 
posterior  staphyloma  is  not  to  be  expected  in  a  myopia  of  less  than  10  D. 
(Schnabel).  1  he  anatomy  and  significance  of  the  conus  are  subjects  of 
discussion.  In  axial  myopia,  the  posterior  segment  of  the  sclera,  par- 
ticularly the  temporal  side,  bulges  outward,  carrying  along  the  attached 
choroid  and  widening  the  aperture  through  which  the  nerve  enters  the 
eye.  At  one  time  it  was  accepted  that  the  backward  excursion  of  the 
sclera  created  the  crescent  either  by  inducing  atrophy  of  the  choroid  or 
dragging  it  away  from  the  edge  of  the  disc.  This  view  has  been  opposed 
on  the  ground  that,   occasionally,   a  crescent,   or  what  looks  like  one,   is 


Fig.  155 — Round,  or  Normally  Shaped 
Globe. 


Fig  155. 


Fig.  156 — Posterior  Bulging  of  the 
Globe  in  Myopia,  causing  its  section  to 
be  Oval  in  Shape. 


Fig.  156. 


MYOPIA.  247 

seen  In  emmetropic  or  hyperopic  eyes.  Furthermore,  it  is  said  that  in 
myopia  a  crescent  may  appear  at  any  point  around  the  disc.  Stilling  con- 
siders the  crescent  to  be  the  white,  inner  wall  of  the  scleral  canal  which 
is  exposed  because,  in  myopia,  the  nerve  is  drawn  obliquely  to  the  temporal 
side  and  the  scleral  foramen  enlarged.  Schnabel  states  that  the  crescent 
or  conus  is  an  imperfect  development  of  the  choroid  and  tissues  around 
the  head  of  the  nerve,  in  fact,  a  slight  coloboma.  Despite  all  theories 
as  to  the  origin  of  the  myopic  crescent,  the  fact  remains  that  its  area  may 
be  enormously  increased  by  atrophy  of  the  choroid  along  its  border. 
The  principal  alteration  found  in  the  anterior  segment  of  a  myopic 
eye  occurs  in  the  ciliary  muscle  in  which  the  circular  fibers  (Miiller's 
muscle)  are  nearly  or  altogether  absent.  Miiller's  muscle  is  employed  in 
the  act  of  accommodation,  but  as  accommodation  is  unnecessary  in  myopia 
it  is  supposed  that  the  fibers  atrophy  from  disuse.  A  thin  poorly  developed 
ciliary  muscle  permits  the  iris  to  recede;  consequently,  the  anterior  chamber 
is  deeper  in  myopia  than  in  emmetropia.  Myopes  have  wide  pupils,  the 
reason  for  which  is  not  altogether  clear;  presumably,  it  depends  on  the 
relation  between  accommodation  and  pupillary  contraction.  The  cornea 
is  supposed  to  be  slightly  flattened  in  axial  myopia,  although  this  has  not 
been  definitely  determined.  The  fundamental  anatomic  alteration  in  axial 
myopia  is  thinning  and  distension  of  the  sclera.  In  1816,  Scarpa  described 
staphyloma  of  the  posterior  segment  of  the  eye,  but  it  was  Arlt  who 
first  recognized  its  connection  with  myopia.  Myopic  bulging  nearly  always 
occurs  within  a  circle  the  center  of  which  is  at  the  outer  edge  of  the  optic 
disc  and  the  periphery  at  the  posterior  border  of  the  oblique  mus- 
cles. In  the  normal  eye  the  posterior  insertion  of  the  inferior  oblique 
is  about  4  mm.  from  the  optic  nerve;  in  myopic  eyes  with  posterior 
staphyloma  this  distance  may  be  increased  to  as  much  as  17  mm.  (Schna- 
bel). The  globe  is  thus  changed  from  a  rounded  (Fig.  155)  to  an  oval 
form  (Fig.  156).  In  the  normal  eye  the  sclera  increases  in  thickness  from 
the  equator  to  the  posterior  pole;  in  mvopia  the  reverse  is  true,  and  the 
sclera  is  thinnest  posteriorly,  where  it  may  be  reduced  to  one-fourth  its 
normal  thickness  (Fig.  160).  In  extreme  cases  the  sclera  may  stretch 
until  it  is  thinner  than  tissue  paper.  In  such  cases  local  areas  may  yield 
to  intraocular  pressure  and  form  abrupt  ectasias,  constituting  the  staphy- 
lo?na  veriim  of  v.  Graefe  (Fig.  157).  These  ectasiae  are  more  apt  to 
occur  on  the  nasal  than  temporal  side  of  the  nerve  and  may  be  recognized 
with  the  ophthalmoscope.  They  seldom  occur  in  eyes  with  less  than 
20  D.  of  myopia.     One  result  of  myopic  distension  is  to  draw  the  retina 


248  THE    FUNDUS    OCULI. 

and  choroid  toward  the  temporal  side  and  over  the  nasal  edge  of  the  nerve. 
In  high  degrees  of  myopia  the  retina  may  thus  be  dragged  to  the  center 
of  the  disc  (  Fig.  158).  The  choroid  also  is  supertracted  but  never  extends 
so  far  o\er  the  nerve  as  does  the  movable  retina.  At  the  same  time 
traction  is  exerted  on  the  scleral  canal,  enlarging  the  aperture  on  the 
temporal  side.  In  the  choroid,  the  vessels  atrophy  and  openings  form  in 
the  membrane  which  are  repaired  by  the  processes  of  granulation  and 
cicatrization,  as  are  the  lesions  of  choroiditis.  The  pigmented  retinal 
epithelium  throughout  the  fundus  is  rarefied,  thus  exposing  the  choroidal 
vessels.  Where  the  choriocapillaris  has  disappeared,  the  retinal  epi- 
thelium perishes  while  the  neighboring  cells  proliferate  and  heap  up  pig- 
ment (Fig.  159).  In  myopia  the  pigment  shows  no  tendency  to  wander 
into  the  inner  lavers  of  the  retina  and  form  branching  figures.  In  areas 
where  the  choroid  is  atrophied  the  rods  and  cones  degenerate  from  malnu- 
trition. The  inner  retinal  layers,  which  are  not  nourished  by  the  choroid, 
are  unaffected  and  the  retinal  vessels  pass  uninterruptedly  across  the 
atrophic  spots.  Unfortunately,  the  macula  falls  within  the  area  of  greatest 
protrusion;  consequently,  it  is  especially  apt  to  suffer  and  pigment  pro- 
liferation, spots  of  degeneration  and  sometimes  holes  may  occur  at  the 
fovea.  Small  degenerated  vessels  are  seen  here  with  the  ophthalmoscope 
as  fine,  zigzag  or  branching  lines.  Macular  hemorrhage  is  not  an  in- 
frequent disaster  in  myopia.  The  vitreous  may  become  liquified  and 
filled  with  floating  opacities.  Although  the  retina  is  not  attached  to  the 
choroid,  it  follows  the  latter  in  its  backward  excursion.  Sometimes,  how- 
ever, a  fluid  vitreous  finds  its  way  behind  the  retina  and  produces  incurable 
detachment.  See  detachment  of  the  retina  (page  173).  Detachment  is 
particularly  to  be  feared  in  high  mvopia,  although  the  lower  grades  are  by 
no  means  exempt,  particularly  if  subjected  to  injury. 

Subretinal  accumulations  of  fluid,  presumablv  choroidal  effusions,  are 
found  in  myopic  eyes  at  points  where  the  choroid  is  destroyed.  Such 
fluid  appears  to  be  connected  with  the  processes  of  repair  rather  than 
with  detachment  of  the  retina   (Fig.   160). 

Progrfssivf.  Myopia.  The  term  progressive  or  pernicious  myopia 
is  applied  to  those  cases  which  are  not  arrested  on  completion  of  body 
growth,  the  eye  continuing  to  elongate  until  the  refraction  reaches  20 
or  30  dioptres.  This  type  differs  from  the  adolescent  or  acquired  myopia 
in  that  it  develops  at  an  earlier  age  and  may  occur  among  the  illiterate  and 
those  who  never  engage  in  close  work.  The  process  of  elongation  is 
more  rapid  and  the  pathologic  changes  more  profound  than  in  the  non- 


Fig.  157  —  Myopia 
with  Local  Ectasia  of 
Sclera,  the  Staphyloma 
J'enn'i    of    Von    Gracfe 


Fig.  158  —  Case  of 
high  Myopia.  The 
Retina  and  Choroid 
have  been  drawn  over 
the  Nasal  Edge  of  the 
Nerve,  toward  the 
Temporal    Margin. 


Fig.  158, 


.\:vopiA.  '  249 

progressive  form.  In  pernicious  myopia  visual  acuity  always  is  lowered 
and,   frequently,  useful  vision  is  lost  from  macular  lesions. 

Stereogram  71.  Progressive  Myopia  (Pernicious  Myopia;  Ma- 
lignant Myopia).  Right  eyeground  of  a  woman  aged  42  years:  Cook. 
The  right  eye  is  myopic  20.  D.;  the  left,  18.  D.  in  the  right  eye,  vision 
with  correction  is  20/200.  Cannot  remember  that  she  ever  saw  well. 
The  fundus  presents  the  ophthalmoscopic  picture  of  pernicious  myopia. 
The  retinal  pigment  has  mostly  disappeared,  leaving  the  choroidal  vessels 
exposed  throughout  the  entire  fundus.  The  retinal  vessels  which  normally 
pass  vertically  upward  and  downward  are  dragged  to  the  temporal  side 
and  straightened.  The  disc  is  surrounded  by  a  wide  white  ring  of  atrophic 
choroid  containing  a  few  specks  of  pigment  and  traces  of  choroidal 
vessels.  Evidently,  this  circumpapillary  ring  is  too  extensive  to  be  regarded 
as  onlv  a  congenital  crescent.  On  both  the  nasal  and  macular  sides  of 
the  nerve  are  several  atrophic  spots  or  cicatrices  bordered  by  pigment, 
identical  in  appearance  with  those  which  follow  the  eruption  of  exudative 
choroiditis.  The  pigment  proliferation  around  the  spots  in  this  fundus  is 
rather  exuberant  for  myopia.  The  softly  outlined  spots  in  the  upper 
field  are  supposed  to  be  gaps  in  the  choroid  in  process  of  repair.  Several 
white  walled,  sclerosed  choroidal  vessels  are  seen  in  which  the  blood  column 
is  reduced  to  a  mere  thread.  Sclerosis  of  the  choroidal  vessels  is  most 
advanced  near  the  disc. 

DlACJNOSls.  The  differentiation  of  myopia  from  other  conditions 
depends,  of  course,  upon  the  refraction  of  the  eye.  It  must  not  be  for- 
gotten, however,  that  choroiditis  from  constitutional  causes  may  occur  in 
a  myopic  eye  and  be  mistaken  for  a  manifestation  of  myopia.  The  choroidal 
lesions  of  myopia  are  comparatively  few  in  number  and  are  limited  to 
the  posterior  pole,  while  those  of  choroiditis  are  numerous  and  widely 
distributed,  but  favor  the  equatorial  region. 

CuRVATi'PL  Myopia.  Myopia  may  be  due  to  increased  curvature 
of  the  cornea  or  surfaces  of  the  lens.  Clinically,  most  if  not  all  cases 
of  corneal  myopia  occur  in  the  diseased  condition  which  results  in  conical 
cornea.  Likewise,  leuticuhir  curvature  myopia  usually  is  due  to  malfor- 
mation or  disease  of  the  lens,  which  results  in  anterior  or  posterior  lenti- 
conus.  Relaxation  or  rupture  of  the  suspensory  ligament,  which  releases  the 
lens  and  permits  it  to  assume  a  spherical  shape  also  causes  myopia.  Under 
the  latter  conditions,  the  younger  the  subject  the  greater  will  be  the  increase 
in  lenticular  curvature.  Hess  has  observed  myopia  develop  in  phthisical 
eyes  also  in  iridocyclitis,  probably  due  to  relaxation  of  the  zonula.     In 


250  THE    FUNDUS    OCULI. 

the  myopia  of  early  senile  and  diabetic  cataract,   it  is  possible  that  the 
curvature  of  the  lens  is  increased    (Parsons). 

Index  Myoplx.  Theoretically,  myopia  may  be  due  to  increase  in 
the  refractive  index  of  the  aqueous,  and  myopia  developing  in  iridocyclitis, 
diabetes  and  jaundice  has  been  attributed  to  this  cause.  Experiments, 
however,  tend  to  dispro\'e  that  the  index  of  the  aqueous  in  these  diseases  is 
ever  sufficiently  increased  to  cause  appreciable  change  in  the  refraction 
(Hess).  J .cut'icular  index  niyopui  is  common  in  commencing  senile  cataract 
and  mav  amount  to  several  dioptres.  The  change  occurs  in  the  nucleus. 
Hirschberg  states  that  the  myopia  of  diabetes  is  due  to  index  changes 
in  the  lens  and  is   independent  of  cataract. 

BihVwgrapliy. 

Arlt.  Krankheitn.   d.    \no.   IT  I,   IS'56:    U.   d.   Einstel.   d.   diop.   Apparates,  1861. 

Donders,   Anamln.   d.   Refr    u.   Accom.,   Wein,   1866. 

Hasner,  Parsons.  Path,  of  the  Eve,  TIL,  92"). 

Hess.    A.   f.   n.   XlXl.   1898. 

Hirschberg,   Deutsch.    m.   Wochenft..   1SF7.    1S91  :     Cent.    f.    A.   XR'.    1890. 

Kepler.  Dioptrice.  etc.,  Ansberg,  1611.  Ref.  in   Parsons.  P.  of  the  Eye,  p.  930. 

JNlauthner,   Op.   Fehler  d.   Ansf.,  \\'ein,   1«76. 

Parsons,  Path,  of  the  Eve,  TIT.  908. 

Pfliiger,  A.   f    O.  XXII, "4,  1876:    Parsons.   IV.  p.   1400. 

Scbnabel  u.  Herrnheiser,  Z.  f.  I-feillv..  XA'I,  1896.     Uber  Staph,  post.,  etc.  189.5.    Norris 

&  Oliver,  III,  p.  395. 
Sraira.    Tratt.    d.   prinrp.    etc..    1*16,    IT,    146 

Schreller,  A.   f.  O.  XXXH,  3,   I'SSG.  C.   f.   A.  XIT,  1888.    Bet.  d.  o.  G.,  1888. 
Stephenson,    Oph.    Rev.,    XI,   l'S92. 
Stillin.ar,  Iv.  Vi.  f.  A.,  XIII.  187.).    .A.,  f.  A.,  XV,  1885.    Beitr.  d.  o.    G.,  1886.    Unt.    u. 

d.  Ents.  d.  Kurtzg.,  1887. 
\ov   Graefe.   A.    f.   O.,   1,  2,   1854. 
Weiss. 


Fig.  159 — High  Myopia  with  Dis- 
appearance of  the  Choriocapillaris 
and  with  Irregular  Massing  of 
Pigment. 


h'lg.   loV. 


Fig.  160 — Thinning  of  the  Sclera  in 
high  Myopia.  An  Accumulation  of 
Fluid  has  taken  place  between  the 
Choroid  and  Retina,  apparently  asso- 
ciated with  the  Process  of  Repair  and 
not  causing  Detachment  of  the  Ret- 
ina, which  is  in   its   Normal   Position. 


Fig.  160. 


Fig.  161 — The  Normal  Filtration 
Angle  C,  cornea;  S,  canal  of 
Schlemm;  P,  pectinate  ligament; 
I,  iris;    C.   B,   ciliary  processes. 


Fig.  161 


Chapter  XIII. 
GLAUCOMA. 

The  essential  element  of  glaucoma  is  increase  in  the  fluid  content 
of  the  globe,  causing  an  abnormal  rise  in  intraocular  pressure.  The 
normal  pressure  within  the  eye  which  produces  touits  biiibi  or  tension,  is 
from  18  to  23  mm.  Hg.  above  that  of  the  atmosphere.  The  maintenance 
of  this  condition,  which  is  essential  to  the  integrity  of  the  eye,  is  largely 
dependent  upon  the  aqueous  humor.  The  aqueous  is  produced  by  the 
ciliary  processes.  The  fluid  enters  first  the  posterior  chamber,  then  passes 
through  the  pupil  into  the  anterior  chamber.  From  the  anterior  cham- 
ber the  excess  of  aqueous  escapes  into  the  general  circulation  by  filtering 
through  the  spaces  of  P'ontana  into  Schlemm's  canal,  which  is  a  plexus  of 
veins  in  the  periphery  of  the  cornea  (Fig.  161).  As  these  drains  are 
situated  in  the  sinus  of  the  anterior  chamber,  this  region  frequently  is 
called  the  (inc/U'  of  filtration. 

The  tension  of  the  globe  being  dependent  upon  the  normal  pro- 
duction and  escape  of  aqueous,  it  is  evident  that  any  obstacle  to  its 
outflow  will  cause  excessive  accumulation  of  fluid  in  the  eye,  increase  intra- 
ocular pressure  and  establish  glaucoma.  If  high  intraocular  pressure 
develops  spontaneously  without  antecedent  eve  disease  or  other  apparent 
cause,  it  is  called  primary  glaucoma ;  on  the  other  hand,  if  increased  tension 
is  due  to  evident  obstruction  to  the  aqueous  current  by  iritic  adhesions, 
displaced  lens,  etc.,  it  is  called  secondary  glaucoma.  Primary  glaucoma 
attacks  both  eyes;  secondary  glaucoma  only  the  eye  which  has  been  pre- 
viously affected. 

Primary  Glaucoma.  This  form  of  glaucoma  occurs  in  those  who 
have  reached  or  passed  the  middle  period  of  life.  The  course  of  the 
disease  is  fairly  uniform  and  divided  into  four  stages  as  follows: 

(1)  .Prodromal  Stage.  During  this  period  the  patient  is  subject 
to  transitory  attacks  of  heightened  intraocular  pressure  during  which  vision 
is  obscured,  usually  more  in  one  eye  than  the  other;  objects  seem  to  be 
enveloped  in  a  fog  and  the  light  of  a  lamp  appears  surrounded  by  a  rain- 
bow colored  ring.  The  attack  is  attended  by  slight  headache  or  neuralgic 
pain  in  the  temple  or  along  the   side  of  the   nose.      On  inspection,   the 

251 


252  THE    FUNDUS    OCULI. 

cornea  is  found  to  be  slightly  clouded  and  there  may  be  some  circum- 
corneal  injection.  The  anterior  chamber  is  shallow.  The  pupil  is  mod- 
erately dilated  and  sluggish  to  the  action  of  light.  Where  predisposition 
to  glaucoma  exists,  but  not  otherwise,  the  attack  is  determined  by  anything 
which  causes  venous  congestion,  as  mental  distress,  depression  of  the  heart, 
dissipation,  loss  of  sleep,  constipation,  etc.  The  foggy  vision  and  halos 
around  lights  are  produced  by  the  "  steamy  "  condition  of  the  cornea. 
Prodromal  attacks  usually  occur  at  intervals  of  weeks.  They  con- 
tinue for  several  hours,  but  always  subside  when  the  patient  sleeps,  if  not 
before.  Exceptionally,  the  attacks  occur  with  great  frequency,  sometimes 
every  morning  on  waking,  but  pass  away  in  the  afternoon.  In  the  in- 
tervals the  eye  is  normal  except  that  the  power  of  accommodation  is  weak- 
ened and  presbyopia  develops  in  advance  of  the  patient's  age.  The  length 
of  the  prodromal  period  may  be  difficult  to  determine,  as  the  initial 
attacks  often  are  slight  and  may  pass  unnoticed.  The  usual  duration  is 
a  few  weeks  or  months.  It  may  be  prolonged  for  years,  in  which  case 
the  eye  gradually  passes  into  a  stage  of  subacute  or  chronic  glaucoma 
without  having  suffered  an  acute  inflammatory  attack. 

(2)  Glaucoma  Evolutinn  (Acute  Inflammatory  Glaucoma;  Glau- 
comatous Crisis).  After  a  longer  or  shorter  prodromal  period,  the  eye 
suddenly  is  attacked  by  an  inflammatory  glaucoma  in  which  all  symptoms 
of  the  prodromal  period  are  greatly  intensified.  These  seizures  are  desig- 
nated according  to  their  severity  as  subacute,  acute  and  fulminating  glau- 
coma. Typical  inflammatory  glaucoma  is  ushered  in  bv  nausea,  vomiting, 
chilly  sensations  and  fe\er.  Excruciating  pains  develop  in  the  head  and 
eye,  often  radiating  through  the  ears  and  teeth.  Not  infrequently  the 
eye  svmptoms  are  so  overshadowed  by  the  intense  headache  as  to  escape* 
observation  and  the  disease  is  mistaken  for  migraine.  Eye  tension  is 
raised,  as  may  be  determined  by  palpation  with  the  fingers.  Vision 
rapidly  fails  and  may  be  reduced  to  perception  of  shadows.  The  intense 
congestive  nature  of  the  disease  is  shown  by  the  external  appearance  of 
the  eve.  The  lids  are  edematous.  Intense  circumcorneal  injection  is 
soon  succeeded  by  bluish,  chemotic  swelling  of  the  conjunctiva.  The  cornea 
is  insensitive  to  the  touch  and  rapidly  becomes  opaque.  The  anterior  cham- 
ber is  shallow.  The  iris  is  congested,  discolored,  and  the  periphery  is 
pushed  forward.  The  pupil  is  dilated  and  immobile  to  light.  The 
pupillary  dilation  may  be  unequal,  in  which  case  the  pupil  is  oval  or  eccen- 
trically situated.  If  seen  at  the  onset  of  the  attack  the  ophthalmoscope 
will  show  pulsation  in  the  retinal  arteries,  but  in  a  short  time  opacity  of 
the  cornea  develops  and  prevents  a  view  of  the  fundus. 


GLAUCOMA.  253 

An  acute  glaucomatous  seizure  is  precipitated  by  the  same  influences 
as  induce  prodromal  attacks,  i.  e.,  anything  which  produces  congestio-^  of 
the  uveal  tract.  An  attack  may  be  due  to  local  causes  as  contusion  of  the 
head  or  slight  injury  to  the  cornea.  An  operation  for  glaucoma  on  one 
eye  may  be  followed  by  acute  glaucoma  in  the  fellow  eye,  presumably  due 
to  mental  depression  incident  to  operation.  Dilation  of  the  pupil  thickens 
the  root  of  the  iris  and  crowds  it  into  the  angle,  thus  blocking  the  outflow 
of  aqueous;  therefore,  the  use  of  a  mydriatic,  especially  atropine,  will 
induce  an  attack  of  glaucoma  in  an  eye  predisposed  to  the  disease. 

An  attack  of  acute  glaucoma  continues  for  some  days  or  weeks, 
when  the  congestive  symptoms  gradually  subside  and  the  eye  again  becomes 
comparatively  well;  the  conjuncti^'al  redness  and  opacity  of  the  cornea 
disappear  and  central  vision  usually  improves  enough  to  enable  the  patient 
to  read.  Inflammatory  glaucoma,  however,  always  leaves  traces  of  its 
presence.  The  vision  is  never  quite  so  good  as  before  the  attack  and 
the  nasal  field  is  contracted.  The  anterior  ciliary  veins,  which  emerge 
through  the  sclera  near  the  cornea,  remain  dilated;  the  anterior  chamber 
is  not  of  normal  depth;  the  iris  is  thinned  and  slate  colored;  the  pupil 
dilated  and  sluggish;  the  eye  tension  is  continuously  above  normal.  Such 
an  eye  is  said  to  present  a  glaucomatous  aspect  (habitus  glaitcomatosiis) . 
When  the  cornea  clears  sufficiently  to  permit  a  view  of  the  fundus,  the 
ophthalmoscope  shows  the  retinal  veins  somewhat  dilated  and  the  arteries 
of  normal  width  or  narrowed.  If  arterial  pulsation  does  not  exist  it  can  be 
produced  by  slight  pressure  on  the  globe  or  by  use  of  a  mydriatic  (which, 
however,  should  never  be  employed  in  primary  glaucoma).  The  optic  disc 
is  cupped  and  the  vessels  are  transferred  to  the  nasal  side.  A  considerable 
period  of  time  may  elapse  before  another  attack  occurs,  during  which  the 
eye  remains  quiet.  Second  and  subsequent  attacks  do  not  differ  from  the 
first,  except  that  they  are  apt  to  be  less  severe.  Explosions  of  the  disease 
recur  with  increasing  frequency,  each  of  which  destroys  some  of  the 
remaining  vision  until  at  last  the  eye  becomes  blind. 

(3)  Glaucoma  Absolutum.  In  this  stage  the  eye  is  hard  as  stone 
and  completely  blind.  The  amount  of  pain  varies;  it  may  be  absent  or  so 
severe  as  to  require  enucleation  of  the  eye.  The  cornea  is  transparent  but 
insensitive  to  the  touch.  The  sclera  is  thinned  and  transmits  the  blue  color 
of  the  underlying  uvea.  The  anterior  ciliary  veins  are  distended  and  tor- 
tuous. The  lens  and  iris  are  advanced,  rendering  the  anterior  chamber 
very  shallow.  The  pupil  is  dilated  and  reacts  slowly  or  not  at  all  to  light. 
The  iris  becomes  greatly  atrophied  and  transillumination  shows  holes  in 


254  THE    FUNDUS    OCULI. 

its  Stroma.  With  the  ophthalmoscope  the  disc  is  seen  to  be  totally  exca- 
vated, the  retina  atrophic  and  the  vessels  more  or  less  degenerated.  Retinal 
hemorrhages  may  occur  and  are  to  be  regarded  as  an  extremely  unfavorable 
symptom.  This  stage  pursues  a  very  slow  course  and  may  continue  for 
years  before   degeneration   is   well  established. 

(4)  Glaucoma  Degenerativiini.  A  glaucomatous  eye  is  classed  as 
degenerative  when  permanent  opacities  appear  in  the  cornea  and  lens. 
In  the  cornea  parenchymatous  opacities  develop,  while  vesicles  and  hyaloid 
deposits  form  on  the  surface.  Opacity  of  the  lens  (cataracta  glaucoma- 
tosa)  always  cievelops.  The  glaucomatous  cataract  is  swollen,  has  a  bluish- 
white  color  and  the  surface  presents  a  silk-like  luster  quite  different  from  the 
dull,  grey,  sectorform  opacity  of  senile  cataract.  In  glaucomatous  cataract 
the  eye  is  blind,  hence,  operation  would  be  useless.  On  the  other  hand, 
ordinary  senile  cataract  may  form  in  a  glaucomatous  eye  with  a  fair 
visual  field,  in  which  case  extraction  miy  be  performed  with  great  benefit  to 
the  patient.  After  a  number  of  years  the  eve  may  atrophy  or  be  lost  from 
perforatmg  ulcer  of  the  cornea.  Extensive  intraocular  hemorrhage,  spon- 
taneous or  following:  slight  injury,  may  occur  and  necessitate  enucleation. 
Occasionally,  such  hemorrhage  may  cause  spontaneous  rupture  of  the  eye- 
ball.   I  have  microscopically  examined  one  such  case*  (Fig  162). 

Glaucoma  Fulminans.  This  term  was  applied  by  Von  Graefe  to 
extremely  violent  cases  of  inflammatory  glaucoma  in  which  vision  is  per- 
manently destroyed  in  a  few  hours  (Fig.   163). 

Chronic  or  Subacute  Glaucoma.  These  terms  are  employed  to 
distinguish  those  cases  of  glaucoma  which  run  their  course  without  at  any 
time  manifesting  acute  congestive  or  inflammatory  symptoms,  although 
the  eye  becomes  injected  and  painful. 

Simple  Glaucoma.  This  remarkable  type  of  glaucoma  is  char- 
acterized by  almost  complete  absence  of  the  concrestive  symptoms  that 
occur  in  the  inflammatory  form  of  the  disease.  Von  Graefe  called  the 
condition  "  amaurosis  with  glaucomatous  excavation  of  the  optic  nerve." 
It  usually  occurs  in  middle  life  but  has  been  seen  in  the  young  and  also 
in  myopes,  who  are  almost  exempt  from  the  inflammatory  type.  Men 
and  women  are  affected  in  about  equal  numbers.  The  disease  always 
attacks  both  eyes,  but  it  often  is  more  advanced  in  one  than  in  the  other. 
Glaucoma  simplex  commences  insidiously,  without  pain  and  exhibits  little 
tendency  to   remissions  or  exacerbations.     Unless  arrested  by  treatment 


*  From  the  service  of  Dr.  J.  \V.  Ingalls,  Brooklyn  Eye  and  Ear  Hospital. 


GLAUCOMA.  255 

it  progresses  uninterruptedly  to  absolute  blindness.  The  eye  tension  is 
continuously  above  normal.  Schiotz,  using  his  tonometer,  found  it  higher 
in  simple,  than  in  congestiv'e  glaucoma.  It  is,  however,  subject  to  fluc- 
tuation; thus,  at  times  increased  tension  cannot  be  detected  with  the  fingers 
but  if  repeated  examinations  are  made  throughout  the  day  hypertension 
will  be  discovered.  The  external  appearance  of  the  eye  usually  is  normal, 
but  at  certain  times,  especially  if  anything  has  occurred  to  cause  uveal 
congestion,  the  cornea  will  be  slightly  clouded,  central  vision  a  little 
lowered  and  a  colored  halo  may  appear  around  lights,  as  occurs  in  the 
prodromal  stage  of  inflammatory  glaucoma. 

Central  \'ision  often  remains  suprisingly  good  until  late  in  the  disease, 
but  the  visual  fields  undergo  great  contraction,  especially  on  the  nasal  side, 
although  sector-like  defects  and  other  variations  are  common.  lorpor 
retinae  often  exists,  and  a  field  which  in  a  bright  light  shows  little  con- 
traction will,  when  illumination  is  reduced,  present  markeci  defects. 

I  he  relationship  of  simple  glaucoma  to  the  inflammatory  type  has 
been  a  subject  of  controversy  and  by  some  it  is  regarded  as  a  peculiar  form 
of  optic  atrophy.  Ihe  identity  of  the  two  conditions,  however,  is  indicated 
by  the  fact  that  simple  glaucoma  may  pass  into  the  chronic  inflammatory 
type  or  an  acute  inflammatory  attack  may  supervene.  Moreover,  the  es- 
sential feature  of  glaucoma — increaseci  tension — exists  in  both  types.  It 
is  probable  that,  in  glaucoma  simplex,  the  very  gradual  development  of  high 
intraocular  pressure,  enables  the  ocular  circulation  to  meet  the  new  con- 
ditions without  inducing  congestion. 

Hi'MORRii  \(;ic  Glaucoma.  The  term  ''  hemorrhagic  glaucoma  "  is 
of  doubtful  utility.  It  has  been  applied  to  a  variety  of  conditions  such 
as  secondary  glaucoma,  which  follow^s  intraocular  hemorrhage,  particu- 
larly, hemorrhagic  retinitis;  also  to  cases  of  simple  or  inflammatory  glau- 
coma occurring  in  a  subject  of  retinal  arteriosclerosis  and,  hence,  par- 
ticularly liable  to  intraocular  hemorrhage.  If  employed  at  all,  the  name 
"hemorrhagic  glaucoma"  should  be  limited  to  the  latter  class  which 
really  are  cases  of  primary  glaucoma  that,  owing  to  the  condition  of  the 
blood  vessels,  possess  a  tendency  to  hemorrhage. 

Ophthalmoscopic  Picture.  In  the  fundus,  the  distinguishing  fea- 
ture of  glaucoma  is  the  characteristic  excavation  of  the  optic  nerve.  Three 
types  of  optic  excavation  are  recognized; 

( 1  )  Physiologic  exca'ualion,  which  is  confined  to  the  center  of  the 
disc  or,  if  it  touches  one  side,  the  margin  Is  not  abrupt  (Stergms.  5-16-27- 
40).     The  lamina  cribrosa  is  in  its  normal  position. 


256  THE    FUNDUS    OCULI. 

(2)  AtropJi'ic  excavation  consisting  of  a  shallow,  dish-like  excava- 
tion of  the  entire  disc  without  undermined  or  abrupt  edges.  There  Is 
no  recession  of  the  lamina  cribrosa    (Stergm.  61). 

(3)  Glaucomatous  excavation  includes  the  entire  disc  and  the  lamina 
cribrosa  is  pushed  backward  (Stergms.  72-73).  Simple  glaucoma  affords 
the  best  opportunity  to  study  the  changes.  In  the  early  stage,  the  disc 
is  pale  and  excavation  commences  on  the  temporal  side  but  gradually  in- 
cludes the  entire  disc.  The  edges  of  a  glaucomatous  excavation  always  are 
precipitous  and  in  many  cases  overhanging.  The  depth  of  the  depression 
continues  to  increase  throughout  the  duration  of  the  disease  and  in  ad- 
vanced cases  measures  6  or  7  dioptres  with  the  ophthalmoscope  (2.  mm.). 
The  nerve  undergoes  atrophy  and  acquires  a  grey  or  greenish  color.  The 
periphery  of  a  glaucomatous  disc  is  shadowed  by  the  walls  of  the  exca- 
vation. The  apertures  in  the  lamina  cribrosa  may  or  may  not  be  visible, 
depending  upon  the  amount  of  adventitious  tissue  in  the  cup.  The  retinal 
vessels  are  displaced  to  the  nasal  side  by  the  early,  temporal  excavation. 
Later  in  the  disease  they  may  move  temporalward.  If  tension  is  high  the 
arteries  pulsate  spontaneously,  but  under  any  conditions  pulsation  is  pro- 
duced by  very  slight  pressure  on  the  eye.  The  veins  are  somewhat  dilated 
and  tortuous,  while  the  arteries  tend  to  become  narrowed.  In  advanced 
cases  all  the  vessels  degenerate.  Atrophy  of  the  retina  and  choroid  occurs 
In  glaucoma.  This  appears  first  around  the  disc  where.  In  addition  to  ex- 
cessive pressure,  the  nerve  fibers  are  subjected  to  traction  by  recession  of 
the  nerve.  This  chorioretinal  atrophy,  known  as  the  glaucomatous  ring, 
forms  a  narrow,  light  colored  border  around  the  nerve,  gradually  increasing 
in  width  as  the  disease  advances.  The  vessels  usually  follow  the  contour 
of  the  excavation,  and  when  the  latter  is  undermined  they  dip  over  the 
brim  and  are  lost  to  view  until  they  reappear  on  the  floor  of  the  depression. 
In  some  cases  the  veins  cling  to  the  walls  of  the  cup,  but  the  arteries  retain 
their  normal  positions,  while  the  nerve  tissue  falls  away  from  them. 

Stereogram  72.  Simple  Glaucoma.  Total  Excavation  of 
Disc.  Left  eyeground  of  a  man,  52  years  of  age.  Vision  has  been  grad- 
ually deteriorating  for  the  past  ten  vears.  Left  eye  has  become  totally 
blind.  Right  eye,  central  vision  is  20/100.  The  visual  field  is  contracted, 
especially  on  the  nasal  side  (Fig.  164).  The  eyes  have  never  been  painful. 
Urine  normal.  Blood  pressure  155  mm.  Hg.  Schiotz  tonometer  re- 
corded the  intraocular  pressure  as  80  mm.  Hg.  The  anterior  chamber 
Is  shallow;  pupil  dilated  and  very  sluggish;  cornea,  lens  and  vitreous  are 
transparent.     The  disc  is  depressed  about    1   mm.    (3.   D.)    and  presents 


Fig.  I(i2. 


i-.s.  103. 


Fig.  1C)2 — Intraocular  Hemorrhage  in  Glaucoma  Perforating  the  Globe  through  the 
Cornea  and  necessitating  Enucleation. 

Fig.   103 — Glaucoma   Fulminans.     Violent   Inflammatory   Glaucoma  with   Rapid   De- 
'struction  of  Vision. 


Fig.  164. 


GLAUCOMA.  257 

the  greenish-grey  color  of  optic  atrophy.  A  wide  glaucomatous  ring 
surrounds  the  excavation.  The  retinal  veins  are  overfull  and  tortuous. 
The  arteries  are  narrowed  and  many  of  the  smaller  branches  have  atro- 
phied. One  artery  on  the  nasal  side  crosses  the  opening  of  the  pit  without 
sinking  until  it  passes  under  the  excavated  edge.  Another  small  artery 
on  the  temporal  side  emerges  from  the  disc,  forms  a  loop  and  returns 
into  the  excavation.  This  is  regarded  as  an  example  of  the  congenital 
anomaly  known  as  an  opticociliary  vessel.  On  the  floor  of  the  excavation 
are  two  small  veins  containing  short  dilations  (Phlebectasias)  .*  Very 
slight  pressure  on  the  globe  produces  pulsation  in  both  arteries  and  veins. 
Although,  in  conformity  with  usage,  the  term  "  inflammatory  "  glau- 
coma has  been  retained  in  this  treatise,  the  name  is  misleading  inasmuch 
as  the  attack  does  not  originate  in  an  inflammatory  process  but  is  a 
violent  congestion  or  strangulation  of  the  tissues  due  to  sudden  increase  in 
intraocular  pressure.  The  noninflammatory  nature  of  a  glaucomatous 
seizure  is  indicated  by  its  sudden  onset  and  by  its  subsidence  when  pres- 
sure is  relieved  by  iridectomy  or  other  measures.  It  is  generally  conceded 
that  increased  tension  is  due  to  some  obstacle  to  the  outflow  of  aqueous, 
but  in  primary  glaucoma  it  has  not  been  definitely  determined  how  this 
originates.  If  the  filtration  angle  in  the  anterior  chamber  is  free  and 
unobstructed  and  the  aqueous  normal,  glaucoma  cannot  occur.  On  the  other 
hand,  obstruction  in  this  filtration  system  immediately  raises  intraocular 
pressure.  Therefore,  it  is  logical  to  assume  that  glaucoma  is  due  to 
blocking  these  drains,  and  practical  inv^estigators  have  endeavored 
to  demonstrate  how  this  occurs.  Obstruction  most  frequently  is  due  to 
approximation  of  the  periphery  of  the  iris  to  the  cornea,  and  in  old  cases 
it  becomes  permanently  fixed  in  this  position  (Figs.  165-166).  In  height- 
ened tension  secondary  to  cyclitis  or  to  retinitis  hemorrhagica,  the  obstacle 
to  filtration  occurs  at  Fontana's  spaces  and  accumulation  of  fluid  in  the 
anterior  chamber  pushes  the  iris  backward  and  deepens  the  chamber  (Fig. 
102).  Primary  glaucoma,  on  the  contrary,  commences  by  advancement 
of  the  iris.  Therefore,  the  primary  cause  of  obstruction  is  whatever  has 
advanced  the  iris,  and  it  is  evident  that  this  must  be  sought  for  behind 
the  anterior  chamber.  The  following  observations  of  Priestly  Smith  are  of 
great  value  and  provide  a  satisfactory  explanation  for  the  majority  of 
typical  cases  of  primary  glaucoma.     Although  the  eye  as  a  whole  ceases 


*For  purpose  of  illustration,  details  at  the  bottom  of  the  excavation  have  been  drawn 
with   greater   distinctness   than   they  possess   when   the   retinal   vessels    are   ophthalmoscop- 
ically  in  focus. 
17 


258  THE    FUNDUS    OCULI. 

to  enlarge  after  adult  life,  the  growth  of  the  lens  continues,  so  that  at 
the  age  of  65  the  lens  is  one-third  larger  than  at  25.  Consequently,  in 
course  of  time  the  lens  becomes  too  large  for  the  eyeball.  The  space 
required  to  accommodate  the  enlarged  lens  is  taken  from  the  anterior 
chamber  by  adv^ancing  the  root  of  the  iris  and  ciliary  processes.  This 
explains  the  way  in  which  the  shallow  chamber  of  old  age  is  produced. 
If,  as  in  hypermetropia,  the  eye  is  below  the  normal  size,  the  continued 
growth  of  the  lens  advances  the  periphery  of  the  iris  beyond  the  limit  of 
safety.  In  such  eyes  swelling  of  the  ciliary  processes  or  dilation  of  the 
pupil  may  push  the  iris  forward  in  contact  with  the  posterior  surface  of 
the  cornea  and  block  the  angle  of  filtration.  The  small  eye  is  especially 
liable  to  glaucoma  whether  or  not  it  is  hypermetropic.  Clinically,  the  size 
of  the  globe  is  determined,  not  by  the  refraction  but  by  the  diameter  of 
the  cornea  which,   as  a  rule,  is  less  than  normal  in  small  eyes. 

Fuchs  savs  that  the  highly  vascular  ciliary  processes  may  swell  and 
block  the  narrow  circumlental  space  in  an  eye  predisposed  to  glaucoma. 
C.  Hess  has  demonstrated  that,  in  adult  life,  the  ciliary  processes  become 
greatly  enlarged.  The  enlargement  is  progressive,  and  in  the  aged  bulbus 
outgrowths  form  which  press  forward  the  iris  and  tend  to  induce  glaucoma. 
Not  only  the  processes  but  the  ciliary  body  itself  often  becomes  cirrhotic 
and  enlarged  in  old  age  (Fig.  167).  Considerable  attention  has  been 
directed  to  the  influence  of  blood  pressure  (vascular  tension)  upon  the 
production  of  glaucoma.  In  the  normal  eye  tension  is  kept  constant  by 
the  aqueous  which  is  regarded  as  a  filtrate  from  the  vessels,  hence,  intra- 
ocular pressure  can  never  exceed  the  blood  pressure  in  the  capillaries, 
otherwise,  aqueous  would  no  longer  be  producecl.  Parsons  says  of  the 
normal  eye.  "  It  is  most  likely  that  this  has  the  highest  capillary  pressure 
of  any  organ  of  the  human  body  when  at  rest."  To  what  extent  local 
capillarv  pressure  varies  in  glaucoma  has  not  been  determineci.  It  has 
been  experimentally  demonstrated,  however,  that  intraocular  pressure 
is  raised  by  anything  which  increases  local  or  general  blood  pressure  in 
either  the  arteries  or  v'eins.  Vascular  constriction  in  distant  parts  also 
increases  intraocular  pressure,  thus,  stimulation  of  the  abdominal  vaso- 
constrictors drives  the  blood  into  the  peripheral  vessels  and  a  rapid  rise  of 
eye  tension  follows.  On  the  other  hand,  section  of  the  spinal  cord  in  the 
cervical  regions  cuts  off  the  abdominal  vasoconstrictors,  and  the  accumula- 
tion of  blood  in  the  great  abdominal  vessels  which  follows  causes  a  decided 
fall  in  the  tension  of  the  eye.  Under  normal  conditions  these  variations  in 
eye  tension  are  compensated  by  changes  m  the  rate  of  filtration,  but  in 


Fig.  165 — Glaucoma  of 
Long  Standing.  The  Root 
of  the  Iris  has  becoiue 
Permanently  Adherent  to 
the  Periphery  of  the 
Cornea,  Completely  Block- 
ing   the    Filtration   Angle. 


Fig.   Uio. 


Fig.  166 — The  same  as  165  but  more 
Highly  Magnified.  C,  cornea;  I,  root  of 
the  Iris;    A,  filtration  angle. 


Fig.    IGU. 


Fig.  167 — Enlargement  of  Ciliary 
Body  and  Ciliary  Processes  in  Ad- 
vanced Life,  Tending  to  Press  the  Iris 
Forward  and  cause  Glaucoma  by  Nar- 
rowing che  Filtration  Angle. 


uL.XLCUMA.  259 

those  suffering  from  vascular  degenerations  it  is  possible  that  the  equilib- 
rium between  the  arterial  and  \enous  systems  of  the  eye  is  sufficiently 
disturbed  to  interfere  with  circulation  in  the  venous  system  of  Schlemm, 
which  drains  the  eye.  Whatever  influence  these  factors  have  in  creating 
a  predisposition  to  glaucoma  is  still  undetermined. 

Changes  in  the  composition  of  the  aqueous  may  retard  filtration.  In 
the  experiments  of  Priestly  Smith,  blood  serum  or  ascetic  Huid  passed  very 
slowly  from  the  anterior  chamber.  The  influence  of  altered  aqueous  upon 
eye  tension  is  clinically  observed  in  subacute  cyclitis,  in  which  the  aqueous  is 
highly  albuminous  and  loaded  with  leucocytes.  Inasmuch  as  the  spaces  of 
Fontana  are  filled  with  leucocytes  and  albuminous  aqueous  is  Rltered  with 
difficulty,  intraocular  pressure  rises.  In  these  cases  the  anterior  chamber 
is  deeper  than  uQrmal  and  the  angle  of  filtration  is  widened.  In  cyclitis 
tension  does  not  rise  to  the  same  height  as  in  congestive  glaucoma,  and 
only  in  chronic,  long  protracted  cases  do  the  deleterious  effects  of  high 
pressure   ensue. 

Pathologists  Avho  examine  manv  eyes  occasionally  encounter  cases  of 
glaucoma  in  which  the  filtration  angle  is  open  and  the  anterior  chamber 
deepened.  Von  Hippel  published  such  a  case  in  which  the  trabecular  of 
the  pectinate  were  thickened  and  the  spaces  filled  with  pigment.  Baques, 
in  a  somewhat  similar  case  following  hemorrhagic  retinitis,  concluded 
that  the  hemorrhages  in  the  retina  and  consequent  destruction  of  retinal 
tissue,  produced  irritating  chemical  substances  which  acted  upon  the  tra- 
becular of  the  pectinate  ligament,  causing  tissue  proliferation  which  closed 
the  spaces.     A  case  of  this  kind  is  described  on  page  154. 

Theoretically,  effusions  into  the  vitreous  which  increase  Its  volume 
should  advance  the  lens  and  iris  and  produce  glaucoma.  This  mav  occur 
in  Intravitreous  bleeding  If  the  hemorrhage  Is  sudden  and  fills  the  eye. 
The  vitreous,  however,  appears  to  accommodate  its  volume  to  the  space  at 
its  disposal  and  the  mere  presence  of  considerable  extraneous  matter 
does  not  raise  the  tension  unless  the  composition  of  the  aqueous  is  so 
changed  as  to  render  its  filtration  difficult.  Increase  in  the  volume  of  the 
vitreous  due  to  obstructions  of  fluids  or  to  pathologic  changes  is  regarded 
by  some  as  a  cause  of  glaucoma,  but  how  this  occurs  remains  to  be 
demonstrated.  If  pressure  in  the  vitreous  exceeds  that  in  the  anterior 
chamber  by  as  much  as  1  mm.  Hg.  the  iris  is  forced  against  the  cornea 
and  the  anterior  chamber  is  obliterated. 

The  etiological  importance  of  choroidal  congestion  in  glaucoma  has, 
undoubtedly,   been  overrated.      It   w^ill   affect   intraocular   pressure   if   ex- 


260  THE    FUNDUS    OCULI. 

cessive  and  suddenly  developed.  Thus,  after  ligature  of  all  the  venae 
vorticosae  eye  tension  rises  to  twice  its  normal  height,  but  the  rise  is 
comparatively  slight  when  but  two  or  three  of  the  veins  are  tied.  After 
the  first  abrupt  rise  tension  commences  to  decline  and  in  a  few  weeks 
becomes  normal,  probably  owing  to  development  of  collateral  anastomoses. 
The  changes  which  follow  ligation  of  the  vorticose  veins  consist  of  intense 
congestion,  transudation  of  highly  albuminous  lymph,  opacity  of  the  vit- 
reous and  cataract. 

A  theory  has  been  advanced  by  Henderson  that  primary  glaucoma 
is  due  to  fibrosis  of  the  trabecular  and  closure  of  the  spaces  in  the  pectinate. 
This  theory  may  apply  to  cases  in  which  the  anterior  chamber  is  deepened, 
as  alwavs  is  the  case  when  the  obstruction  originates  in  the  filtration  spaces. 

Other  theories  held  to  explain  primary  glaucoma  are  choroiditis  with 
excessive  serous  exudation;  anterior  iridocyclitis;  variations  in  osmotic 
pressure  in  the  vitreous;  hardening  of  the  sclerotic  around  the  nerve, 
thereby  obstructing  the  outflow  of  lymph.  It  is  improbable  that  obstruction 
in  the  lymph  channels  about  the  optic  nerve  and  around  the  venae  vorticosae 
can  influence  eye  tension  so  long  as  drainage  through  the  anterior  chamber 
is  free  and  unobstructed.  Possibly,  their  closure  may  exert  some  influence 
on  the  vitreous.  Nerve  irritation  was  suggested  by  Donders  as  a  cause 
of  glaucoma.  Eye  tension  can  readily  be  raised  and  lowered  by  irritation 
or  inhibition  of  nerves,  but  as  this  always  is  accompanied  by  a  correspond- 
ing rise  and  fall  in  local  blood  pressure  it  may  be  regarded  as  a  compen- 
satory process.  High  blood  pressure  Is  unfavorable  in  glaucoma,  but 
the  influence  of  arteriosclerosis  upon  the  dev^elopment  of  glaucoma  has 
not  been  determined.  It  appears  reasonable  that  obstruction  might  arise 
from  vascular  degeneration  in  the  efferent  vessels  which  drain  the  anterior 
chamber.  Von  Graefe  first  called  attention  to  the  influence  of  heredity 
in  glaucoma  and  pointed  out  the  curious  fact  that  in  hereditary  glaucoma 
there  is  a  marked  tendency  for  the  disease  to  anticipate,  /.  e.,  to  appear 
in  a  descendant  at  an  earlier  age  than  In  the  ancestor.  Thus,  the  pro- 
dromata  which  appeared  in  a  grandparent  at  60  appear  in  a  grandchild 
at  30  or  40.  Hereditary  glaucoma  usually,  but  not  Invariably,  is  exceed- 
ingly chron'c  in  Its  course.  Among  the  constitutional  conditions  which 
favor  the  development  of  glaucoma  gout  is  most  prominent. 

Secondary  Glaucoma.  The  course  of  the  aqueous  between  the 
ciliary  processes  and  pectinate  ligament  may  be  mechanically  obstructed  by 
various  conditions.  The  most  common  are  the  several  forms  of  iritic  ad- 
hesions.    If  the  exudate  in  iritis  Is  markedly  fibrinous  it  adheres  to  the 


Fig.  168 — Membrane  (M)  Originating 
from  Inflammation  of  the  Iris  (I)  and 
Closing  the  Pupil  (not  shown  in  the  fig- 
ure).    Occliisio  Piipillae. 


Fig.  168. 


Fig.  169— Iris  Bombe.  The  Root  of  the 
Iris  (I)  is  Adherent  to  the  Cornea  (C)  and 
its    Pupillary    Margin   to  the   Lens    (L). 


Fig.   109. 


Fig.  170  —  Total  Posterior 
Synechia  with  Advancement  of 
the  Lens  from  Pressure  Cre- 
ated  in   the   Posterior   Chamber. 


Fig.   170. 


Fig.  171. 

Fig.  171 — Cyst  of  the  Pars  Ciliaris  Retin?e  in 
a  Degenerated  Eye.  The  Iris  is  Adherent  to 
the  Cornea,  thus  Blocking  the  Filtration  Angle 
and  Causing  Glaucoma.  It  is  also  Adherent  by 
a  broad,  flat  surface,  to  the  Lens,  (total  pos- 
terior synechia). 


Fig.  172 — Total  Anterior  Synechia  with  An- 
terior Staphyloma,  Causing  Secondary  Glau- 
coma. The  Optic  Disc  shows  Glaucomatous 
Cupping. 


Fig.  ITo — Secondary  Glau- 
coma Caused  by  Lens  in 
Contact  w  i  t  h  M  a  r  g  i  n  a  1 
t'orneal  Perforation,  Block- 
ing  the   Filtration  Angle. 


Fig.  173 


Fig.  174. 


Fig.  174 — Epithelial  Cyst  Formation.  Continuity  of  Ingrowing  Epithelium  has  been 
Severed  by  Union  of  Corneal  Wound  Isolating  Cells  in  Iris.  S,  surface  epithelium;  P, 
corneal  cicatrix  marking  line  of  perforation;    E,  epithelial  cyst  in  iris;    1,  iris. 


Fig.  175. 


Fig. 175 — Anterior  Chamber  Obliterated  by  Inflammatory  Adhesions  between  Iris 
and  Cornea  except  a  Cystic  Space  (A)  lined  with  Epithelium.  C,  cicatricial  cornea;  I, 
iris;    L,  lens  matter. 


Fig.  17G. 


Fig.  176 — Glaucoma  Produced  by  Epithelium 
Proliferating  into  and  Blocking  the  Filtration 
Angle.  S,  surface  epithelium;  C,  cornea;  B,. 
ciliary  body;     E,   filtration  angle. 


Fig.  177 — Spontaneous  Dislocation  of  Lens 
(L)  into  the  Antcri  ;r  Chamber,  Causing  Glau- 
coma. 


Fig.  177. 


(JLALCO.MA.  261 

anterior  surface  of  the  iris  and  sometimes  also  to  the  anterior  surface  of  the 
lens.  In  the  latter  case  a  membrane  which  closes  the  pupil  may  organize 
{occlusio  piipULr)  (Fig.  168).  I  his  condition  produces  glaucoma  in  which 
the  iris  and  sometimes  also  the  lens  are  carried  forward  against  the  cornea. 

The  pupillary  margin  of  the  iris  may  be  adherent  to  the  anterior 
surface  of  the  lens  {posterior  antmlar  synechia) .  The  aqueous  being  unable 
to  pass  through  the  pupil  into  the  anterior  chamber  causes  the  iris  to 
bulge  forward  producing  the  condition  known  as  irts  hombe   (Fig.   169). 

In  total  posterior  synechia  broad,  flat  adhesions  exist  between  the 
lens  and  iris.  The  lens  usually  is  advanced  against  the  cornea  by  the  pres- 
sure In  the  posterior  chambers  of  the  eye   (Figs.   170-171). 

Total  anterior  synechia  usually  follows  a  perforating  wound  or  ulcer 
of  the  cornea.  With  occurrence  of  perforation  the  aqueous  escapes,  so  that 
the  iris  falls  in  contact  with  the  posterior  surface  of  the  cornea  and  the 
anterior  chamber  disappears.  Under  favorable  conditions  the  perforation 
closes  and  the  anterior  chamber  is  restored,  new  aqueous  being  elaborated. 
Usually,  howe\'er,  more  or  less  extensive  adhesions  occur  between  the 
iris  and  cornea.  If  the  pupil  is  included  in  the  adhesion,  secondary  glau- 
coma and  bulging  of  the  cornea  (staphyloma)  follow.  The  protrusion 
may  Involve  either  the  whole  or  only  a  part  of  the  cornea  (Fig.  172). 
In  every  instance  the  protruded  portion  Is  composed  of  opaque  cicatricial 
tissue  which  develops  from  the  iris.  Sometimes  the  lens  Is  swept  into  a 
corneal  perforation  and  remains  to  block  the  pupil   (Fig.   173). 

After  perforating  wounds  of  the  cornea,  the  corneal  epithelium  may 
proliferate  along  the  wound  into  the  iris  and  form  a  cyst  (Fig.  174),  or 
It  may  line  the  anterior  chamber  including  the  pectinate  ligament  (Figs. 
175-176).  In  either  event  the  angle  of  filtration  becomes  blocked  and 
secondary  glaucoma  develops. 

As  a  result  of  a  blow  on  the  eye,  the  lens  may  be  driven  forward 
and  crush  the  iris  into  the  angle  of  filtration  where  It  remains  and  produces 
secondarv  glaucoma  (Fig.  132). 

Spontaneous  dislocation  of  the  lens  into  the  anterior  chamber  totallv 
obstructs  the  drainage  system  of  the  anterior  chamber  and  results  In  a  pure 
pressure  glaucoma  disassociated  from  inflammation  or  trauma  (Figs.  177- 
178).  Traumatic  dislocation  of  the  lens  into  the  anterior  chamber  also 
results  in  glaucoma,  but  often  is  associated  with  other  injuries.  After 
perforating  wounds  of  the  lens,  as  after  the  operation  of  discission,  the 
lens  matter  swells  and  may  cause  hypertension  either  by  mere  increase  in 
size  or  by  loose  lens  matter  blocking  the  pupil  or  the  angle  of  filtration. 


262  THE    FUNDUS    OCULI. 

After  cataract  extraction  about  one  per  cent  of  the  cases  develop  glau- 
coma (Knapp).  This  complication  may  occur  at  any  time  within  a  year 
or  two  after  closure  of  the  wound.  Risley  describes  a  case  of  glaucoma 
which  developed  in  an  aphakic  eye  sev^enteen  years  after  extraction.  After 
removal  of  the  lens,  if  a  large  quantity  of  cortical  matter  is  left  behind 
it  may  block  the  filtration  angle  or  excite  sufficient  ciliary  congestion  and 
swelling  to  advance  the  periphery  of  the  iris.  Probably,  the  inost  frequent 
cause  of  hypertension  after  extraction  is  incarceration  and  retention  of  the 
lens  capsule  or  iris  in  the  wound.  Sometimes  vitreous  is  prolapsed  and 
fills  the  anterior  chamber  to  the  exclusion  of  the  aqueous,  in  which  case 
it  may  effectually  block  drainage.  In  these  cases  it  is  doubtful  if  the  com- 
position of  the  ^•Itreous  is  normal.  A  transparent  capsular  or  inflammatory 
membrane  may  completely  occlude  the  pupil  and  cause  glaucoma  which 
abates  when  the  pupillary  space  is  incised  with  a  knife  needle.  In  other 
cases  the  pupillary  membrane  is  ciense  and  readily  discovered  (Fig.  179). 

Growths  in  the  ciliary  body  may  advance  the  iris  and  mechanically 
close  the  angle  but  in  other  cases  a  neoplasm  may  draw  the  iris  away 
from  the  cornea  and  open  the  angle,  yet  glaucoma  develops   (Fig.   180). 

In  sarcoma  of  the  choroid,  glaucoma  appears  long  before  the  tumor 
reaches  the  ciliary  body,  but  the  eye  usually  does  not  become  glaucomatous 
until  the  retina  is  extensively  detached  (Fig.  181).  Several  factors  con- 
tribute to  the  development  of  glaucoma  in  these  cases:  (1)  Altered  vit- 
reous which  mechanically  pushes  forward  the  lens  and  periphery  of  the 
iris;  (2)  Irritating  effect  of  tumor  juices  upon  the  trabeculas  of  the 
pectinate;  (3)  Blocking  the  spaces  of  Fontana  with  leucocytes,  pigment 
and  debris;  (4)  Pressure  of  the  tumor  on  a  vortex  vein  (of  doubtful 
influence) . 

Evidently,  it  is  impossible  to  describe  all  methods  by  which  the  filtra- 
tion angle  may  be  obstructed.  The  following,  however,  are  not  very 
unusual  results  of  perforating  wounds;  ( 1 )  cystic  degeneration  of  iris  and 
ciliary  body  (Figs.  171-182)  ;  (2)  deposit  by  the  endothelial  cells  of  the 
cornea  and  iris  of  a  cuticular  material  resembling  Descemet's  membrane, 
in  the  angle  of  filtration  (Fig.  183). 

Morbid  Anatomy.  The  parenchymatous  haziness  of  the  cornea 
in  glaucoma  is  due  to  disarrangement  of  its  parts.  The  same  effect  can 
be  produced  in  a  freshly  enucleated  eye  by  compression.  In  the  epithelial 
layer  the  cloudiness  is  caused  by  edema;  the  fluid  accumulates  between  the 
epithelial  cells  and  on  the  surface  of  Bowman's  membrane.  This  edema 
probably  is  caused  by  interference  with  the  circumcorneal  circulation.     In 


Fig.  178 — Glaucoma  Following  Spjiitancous 
Dislocation  of  the  l.cn-^  into  the  Anterior  Cham- 
ber. Although  Pre->ure  ha.s  caused  Ectasia  o', 
the  Sclera,  the  Disc  does  not  present  a  Typ'cal 
Glaucomatous  Clipping.     Sec  also  Fig.  177. 


Fig.   ITS. 


Fig.  179 — Glaucoma  Follow- 
ing E.xtraction  of  Cataract  and 
due  to  a  Dense  Pupillary  Mem- 
brane. 


Fig.  180 — Sarcoma  of  the 
Choroid  Drawing  the  Iris  awa}' 
from  the  Cornea.  The  Filtration 
Angle  is  open.  S,  sarcoma;  F 
filtration  angle. 


Fig.  180. 


Fig.  ISl — Sarcoma  of  the  Choroid,  with 
Retinal  Detaclinient,  Causing  Secondary 
Glaucoma.  The  Tumor  has  not  Involved 
the  Ciliary  Body. 


Fig.  181. 


Fig.  182— Cystic  Degeneration  of  the  Iris 
and  Ciliary  Body  Blocking  the  Filtration 
Angle. 


Fig.   182. 


Fig.  183 — Filtration  Angle  Blocked 
by  Membrane  Resembling  that  of 
Descemet  and  Derived  from  the 
Endothelial  Cells  of  the  Cornea  and 
Iris. 

A,  filtration  angle. 

I,  iris. 

D,  Descemet's  membrane. 

P,  newly  formed  membrane. 


Fig.  las. 


Fig.  184 — Extreme  Atrophy  of  the  Iris 
(I)    and    Ciliarj-   Bodj-  in   Old    Glaucoma. 


Fig.  184. 


Fig.    185 — •  Ciliarj'    Staphyloma.      C, 
cornea;    C.    B..    ciliary    body;    L,    lens. 


Figr.  185. 


Fig.    186 — Intercalar\-   Staphyloma. 


Fig.  18G. 


GLAUCOMA.  263 

nearly  all  cases  submitted  to  microscopic  examination  the  angle  of  filtra- 
tion is  obliterated  by  adhesion  of  the  iris  to  the  cornea.  In  the  early  stage 
the  iris  is  pushed  against  the  cornea  by  swelling  of  the  ciliary  processes 
but  recedes  when  the  attack  subsides.  In  course  of  time,  howev^er,  per- 
manent adhesions  form  between  the  iris  and  cornea,  thus  producing  the 
shallow  anterior  chamber  of  glaucoma  absolutum  (Fig.  165).  In  the 
early  stage  there  is  inflammatory  edema  of  the  iris  and  ciliary  body,  but  in 
the  late  stages  the  iris  becomes  too  atrophic  to  manifest  inflammatory  phe- 
nomena. Where  the  iris  is  attached  to  the  cornea  nothing  remains  but  a 
line  of  pigment.  In  the  free  portion,  the  delicate  stroma  of  the  iris  is 
replaced  by  fibrous  tissue.  The  blood  vessels  are  extensively  degenerated 
or  entirely  obliterated.  The  muscular  fibers  undergo  hyaloid  degenera- 
tion. The  pigment  cells  disappear  from  the  stroma,  causing  the  iris  to 
change  its  color.  The  posterior  pigmented  layer  is  well  preserved  and  as 
the  stroma  contracts  this  layer  is  dragged  forward  and  over  the  anterior 
surface,  so  that  the  pupil  is  surrounded  by  a  black  ring  constituting  the  con- 
dition known  as  crn-opioii  of  the  uveal  pigment.  The  ciliary  body  and  iris 
become  flattened  from  atrophy  until  in  very  old  cases  they  are  represented 
by  only  a  line  of  pigment  (Fig.  184).  At  last  the  ciliary  processes  disap- 
pear, aqueous  is  no  longer  produced  and  the  globe  softens.  Changes  in  the 
choroid  correspond  to  those  in  other  parts  of  the  uvea.  At  first  there  is 
inflammatory  edema  with  exudation  of  coagulable  serum,  and  in  the  late 
stages  extreme  atrophy.  Progressive  atrophy  of  the  retina  occurs  com- 
mencing on  the  temporal  side. 

The  sclera  becomes  thinner  and  thinner  and  at  last  yields  at  its 
weakest  part,  namely,  around  the  anterior  ciliary  vessels  and  at  the  sclero- 
corneal  junction,  where  it  has  been  weakened  by  congestion  and  inflam- 
mation. If  the  bulging  occurs  ov^er  the  ciliary  body  it  is  called  ciliary 
staphyloma  (Fig.  185).  If  it  takes  place  immediately  in  front  of  the 
ciliary  body,  it  is  termed  intercalary  staphyloma  (Fig.  186).  Equatorial 
staphyloma  occurs  around  the  vena  vorticosas  (Fig.  187).  Hemorrhage 
may  occur  in  this  region  from  venous  occlusion.  Flattening  of  the  cornea 
results  from  stretching  of  the  cornea  and  sclera  at  their  junction. 

Excavation  of  the  nerve  head  in  glaucoma  is  generally  attributed 
solely  to  increased  intraocular  pressure,  the  nerve  yielding  because  the 
scleral  fibers  are  least  numerous  at  this  point.  This  apparently  simple 
explanation  does  not  altogether  conform  to  the  course  of  the  disease  or 
to  the  pathologic  findings,  which  appear  to  indicate  that  the  lamina  does 
not  yield  to   excessive   pressure   until   the   nerve   has   been   weakened   by 


264  THE    FUNDUS    OCULI. 

atrophy  and  disappearance  of  the  disc  fibers.  Although  the  scleral  fibers 
are  least  numerous  at  the  lamina,  this  region  is  heavily  reinforced  by  the 
nerve  structure  and  is  by  no  means  the  least  resistant  portion  of  the  eye 
tunics,  as  is  demonstrated  by  artificially  produced  pressure  from  injections 
cf  fluid  and  by  cases  of  pure  pressure  glaucoma  due  to  dislocation  of  the 
lens  into  the  anterior  chamber  (Figs.  177-178).  In  estimating  the  value 
of  observations  on  this  subject,  it  is  important  to  separate  cases  in  which 
aqueous  obstruction  occurred  suddenly  from  those  in  which  it  developed 
slowly  or  intermittently.  In  the  latter  class  excavation  is  extreme.  Schna- 
bel  regards  the  excavation  as  due  to  neuritic  atrophy,  which  commences 
by  formation  of  cavities  in  the  nerve  tissue  which  are  supposed  to  fuse 
and  form  the  glaucomatous  excavation.  In  all  the  early  glaucomas  which 
I  have  examined  microscopically,  pit-like  depressions  were  found  in  which 
the  nerve  axons  had  disappeared,  but  the  neuroglia  remained  (Fig.  188). 
Excavation  of  the  disc  commences  on  the  temporal  side  (Figs.  189-190). 
Continued  high  pressure  soon  leads  to  total  excavation  of  the  disc.  Optic 
atrophy  precedes  or  keeps  pace  with  the  excavation  and  in  course  of  time 
all  nerve  fibers  are  destroyed.  The  glaucomatous  cup  usually  is  filled 
only  with  vitreous  and  lined  by  atrophic  nerve  fibers  (Fig.  191).  In 
some  cases,  however,  it  contains  a  large  amount  of  newly  formed  connective 
tissue  (Fig.  192).  This  adventitious  tissue  may  be  quite  vascular  (Fig. 
193)  and  when  congested  present  an  ophthalmoscopic  picture  simulating 
neuritis.  Microscopic  sections  of  glaucomatous  eyes  frequently  show 
fibrous  elevations  projectlno;  from  the  floor  of  the  excavation;  they  often 
contain  nearly  or  completely  obliterated  blood  vessels   (Fig.   194). 

BuPHTHALMUS  (Infantile  Glaucoma;  Hydrophthalmus :  Ox  Eye). 
Buphthalmus  is  the  glaucoma  of  infancy.  In  childhood  the  sclera  is  far 
more  distensible  than  in  adult  life  and  when  subjected  to  increased  intra- 
ocular pressure  it  vields  uniformly.  The  entire  globe  is  enlarged  including 
the  cornea  which  may  form  a  globular  protuberance  (keratoglobus)  or 
it  mav  be  stretched  and  flattened.  The  anterior  chamber  is  very  deep. 
Buphthalmus  resembles  simple  glaucoma  in  the  entire  absence  of  con- 
gestive and  inflammatory  phenomena.  In  both  conditions  this  may  be 
ascribed  to  the  slow  and  uniform  development  of  high  tension.  In  the 
early  stages  the  iris  appears  normal  but  gradually  it  changes  color  and 
finally  becomes  highly  atrophic.  The  lens  remains  clear  until  distension 
and  degeneration  are  well  advanced  when  it  becomes  cataractous  from 
malnutrition.  Subluxation  of  the  lens  may  occur  from  stretching  and 
rupture   of   the    zonular   fibers.      The   lens    recedes    and  no    longer    sup- 


Fig.  187. 
Fig.  187 — Equatorial  Staphyloma. 


Fig.   188. 


Fig.    188 — Early    Glaucoma   with    Pit-like    Depressions    on    the    Disc,    from    which    the 
Nerve  Axons  have  Disappeared,  the  Neuroglia  Remaining. 


Figs.  189  and  190 — Early  Glau- 
comatous Cupping  of  the  Disc. 
The  Excavation  Commences  on 
the  Temporal  side. 


Fig.  190. 


Fig.  191  —  Glaucomatous  Cupping, 
Later  Stage.  Complete  Excavation. 
The  Optic  Nerve  is  Atrophic. 


Fis-   i'-'J^- 


GLAUCOMA.  265 

ports  the  iris  so  that  the  hitter  trembles  when  the  eye  is  moved 
(iridodonesis).  The  retina  and  choroid  undergo  atrophy.  Although 
the  antero-posterior  diameter  of  the  globe  is  greatly  increased,  the 
refraction  is  not  always  myopic  and  the  myopia,  when  present,  is  not 
usually  of  high  degree.  This  is  explained  by  the  backward  displacement 
of  the  lens.  Buphthalmus  frequently  occurs  in  a  family  in  which  a  parent 
and  several  children  are  affected  by  this  or  some  other  congenital  ocular 
anomaly. 

The  obstruction  to  escape  of  aqueous  is  situated  in  the  anterior  cham- 
ber and  appears  to  be  due  to  arrested  development,  the  iris  remaining  as 
in  fetal  life,  in  contact  with  the  cornea  at  the  angle  of  the  anterior  chamber 
(Fig.  195).  rhere  is  also  undue  persistence  of  the  fetal  pectinate  liga- 
ment. These  or  other  anomalies  in  the  filtration  system  have  been  re- 
ported in  the  few  cases  which  have  been  examined  microscopically.  It  has 
been  experimentally  demonstrated  that  the  rate  of  filtration  from  buph- 
thalmic  eyes  is  subnormal. 

Stereogram  73.  Glaucoma  wrrn  Adnaxced  Arteriosclerosis 
(So-called  Hemorrhagic  Glaucoma).  Early  Glaucomatous  Exca- 
vation OF  Nerve.  Right  eyeground  of  a  man  72  years  of  age. 
Has  general  arteriosclerosis.  Both  eyes  are  affected  with  glaucoma  sim- 
plex. Two  weeks  ago  a  "  blur  "  suddenly  appeared  before  the  right  eye. 
Vision,  10/200:  tension,  taken  with  the  finger,  is  plus.  The  temporal  side 
of  the  nerve  presents  a  typical  glaucomatous  excavation.  A  light  halo  in 
the  adjoining  fundus  marks  commencing  choroidal  atrophy.  The  exca- 
vation extends  to  the  temporal  edge  of  the  disc  which  is  slightly  under- 
mined. The  nasal  side  of  the  depression  is  precipitous,  but,  has  not  yet 
extended  to  the  margin  of  the  nerve.  The  floor  is  grey  in  color  and  dis- 
tinctly shows  the  perforations  in  the  lamina.  The  main  retinal  vessels 
are  displaced  to  the  nasal  side  of  the  nerve.  The  ascending  and  descend- 
ing retinal  arteries  are  sclerosed  as  shown  where  they  cross  and  obscure 
underlying  veins.  Numerous  small,  striate  hemorrhages  are  scattered 
throughout  the  macular  region  and  along  the  supratemporal  vessels. 

Ten  days  after  the  above  picture  was  secured,  extensive  hemorrhage 
occurred  into  the  vitreous  and  blood  appeared  in  the  anterior  chamber. 
In  two  weeks  inflammatory  glaucoma  developed  and  the  eye  was 
enucleated. 

Microscopic  Examination  In  the  nerve,  both  central  vessels  were 
extensively  degenerated  and  the  artery  was  nearly  closed  by  endarteritis 
proliferans.     The  retinal  vessels  presented  the  usual  changes  of  arterio- 


266  THE    FUNDUS    OCULI. 

sclerosis.  The  entire  retina  was  filled  with  deep  and  superficial  hemor- 
rhages. The  optic  disc  was  excavated  on  the  temporal  side  (Fig.  190). 
The  lamina  cribrosa  was  not  displaced. 

Diagnosis.  Acute  inflammatory  glaucoma  is  an  emergency  disease 
which,  if  recognized,  is  readily  relieved,  while  a  mistake  in  diagnosis  usu- 
ally results  in  destruction  of  vision.  Notwithstanding  the  vital  necessity 
of  recognizing  this  not  uncommon  disease,  it  seldom  is  diagnosticated  by 
the  general  practitioner,  in  fact,  a  large  proportion  of  glaucoma  cases  which 
enter  our  clinics  have  had  the  condition  aggravated  or  even  \nsion  de- 
stroyed by  the  use  of  atropine  which  has  been  dropped  into  the  eye  for  a 
supposed  iritis.  During  a  glaucomatous  seizure,  the  eye  condition  may  be 
masked  by  violent  pain  throughout  the  distribution  of  the  fifth  nerve  and 
the  disease  is  regarded  as  an  attack  of  megrim.  Periodic  headache  or 
megrim  occurs  with  considerable  regularity  from  childhood  throughout 
life,  although  the  intervals  of  attack  may  grow  longer  after  forty-five. 
Glaucoma,  on  the  other  hand,  seldom  appears  before  forty,  after  which 
attacks  occur  with  increasing  frequency.  The  aura  wTiich  precede  megrim 
and  enable  the  patient  to  foretell  a  seizure,  are  absent  in  glaucoma.  The 
mistake  of  regarding  glaucoma  as  megrim,  however,  is  less  dangerous 
than  that  frequently  made  of  diagnosticating  and  treating  the  disease  as 
iritis.  It  is  very  important  to  differentiate  these  two  diseases,  inasmuch  as 
atropine,  which  is  beneficial  in  iritis,  is  destructive  in  glaucoma.  The  ap- 
pearance of  the  iris  is  diagnostic;  in  glaucoma  it  is  pushed  forward, 
especially  at  the  periphery,  and  the  pupil  is  dilated,  oval  and  immobile  to 
light.  In  iritis  the  iris  is  not  advanced,  the  pupil  is  contracted,  irregular 
and  may  be  filled  with  exudates.  In  glaucoma  the  iris  is  free,  while  in 
iritis  it  usually  is  bound  to  the  lens  bv  synechia.  The  cornea  in  glaucoma 
is  steamy  and  insensitive  to  the  touch;  in  iritis  it  is  clear  and  sensibility  is 
not  affected.  In  iritis  the  pain  develops  slowly  and  is  worse  at  night;  in 
glaucoma  it  de\'elops  rapidly,  is  continuous  and  more  severe  than 
in  iritis.  Vision  is  lowered  in  iritis,  but  may  be  abolished  in  acute 
glaucoma.  The  deciding  symptom  in  doubtful  cases  is  eve  tension,  which 
is  normal  in  uncomplicated  iritis,*  but  always  increased  in  glaucoma.! 

Iridocyclitis,  especially  of  syphilitic  origin,  may  develop  violent  noc- 
turnal exacerbations  attended  by  vomiting  and  radiating  pains  in  the 
supraorbital  region.     During  such  an  attack  eye  tension  may  be  increased 


*  ft   is  understood  that  some  irritation  of  the  ciliary  body  always  accompanies  iritis. 
The  term  "  uncomplicated  iritis  "  refers  to  cases  presenting  no  clinical  evidence  of  cyclitis. 
t  Bear  in  mind  that  secondary  glaucoma  may  be  caused  by  iritic  adhesions. 


GLAUCOMA.  267 

and  the  condition  so  closely  simulate  inflammatory  glaucoma  that  differ- 
entiation  is   difficult.      These   cases   are    recognized   as   iridocyclitis   by — 

(1)  The  history,  which  in  iridocyclitis  is  that  of  a  slowly  developed 
inflammation. 

(2)  Contracted  pupil  and  presence  of  iritic  adhesions. 

(3)  In  the  secondary  glaucoma  of  iridocyclitis  the  anterior  chamber 
usually  is  of  normal  or  increased  depth,  while  in  primary  glaucoma  it  al- 
ways is  shallow. 

(4)  The  presence  of  punctate  deposits  on  the  posterior  surface  of 
the  cornea  and  on  the  lens.  This  is  the  most  important  differential  symp- 
tom. These  deposits  consist  of  conglomerations  of  leucocytes  and  pig- 
ment from  the  ciliary  body  which,  following  the  direction  of  the  aqueous 
current  are  deposited  upon  the  lens,  iris  and  cornea.  As  a  result  of  grav- 
itation they  tend  to  settle  upon  the  lower  portion  of  the  cornea  and  should 
be  sought  for  in  this  situation  by  oblique  illumination.  When  the  exudate  is 
abundant  it  fills  the  sinus  of  the  chamber  below,  forming  a  hypopyon.  The 
presence  of  even  a  few  punctate  deposits  on  the  posterior  surface  of  the 
cornea  is  proof  that  the  disease  is  not  glaucoma  but  iridocyclitis. 

(5)  In  cyclitis  with  increased  tension  some  anesthesia  of  the  cornea 
may  exist,  but  never  is  so  complete  as  in  inflammatory  glaucoma. 

The  ophthalmoscopic  picture  of  glaucoma  is  best  obtained  in  the 
simple  and  subacute  forms  of  the  disease,  also  in  the  inflammatory  form 
during  the  intervals  of  attack.  Early  glaucomatous  excavation  frequently 
requires  differentiation  from  physiologic  excavation  of  the  nerve.  In 
glaucoma  the  nerve  is  grey  and  atrophic  and  the  excavation  extends  to  the 
very  edge  of  the  nerve,  which  is  precipitous  or  undermined,  while  in 
physiologic  excavation  the  depression  does  not  extend  to  the  edge  of  the 
nerve.  In  the  oblique,  funnel-shaped  physiologic  excavations  (Stergm.  5) 
the  disc  gradually  slopes  from  the  edge  into  the  cup  in  a  manner  quite  dif- 
ferent from  the  abrupt  edge  of  a  glaucomatous  pit. 

The  ease  with  which  pulsation  is  induced  in  a  glaucomatous  eye  by 
pressure  with  the  finger  is  diagnostic. 

A  small  coloboma  of  the  nerve  may  present  a  superficial  resemblance 
to  glaucoma  (Stergm.  9),  but  is  readily  differentiated  by  its  congenital  and 
non-progressive  character  and  by  the  otherwise  normal  condition  of  the 
eye. 

Intraocular  tumors,  especially  sarcoma  of  the  choroid,  induce  a  sec- 
ondary inflammatory  glaucoma  which  requires  to  be  diagnosticated  from 
the  primary  form.  Sarcoma  is  suspected  if  the  eye  was  blind  before  the 
attack  of  glaucoma  and  especially  if  the  vision  disappeared  slowly  and 


268 


THE    FUNDUS    OCULI. 


painlessly.  Sarcoma  occurs  in  one  eye;  glaucoma  in  both  eyes,  therefore, 
bilateral  glaucoma  is  evidence  against  the  presence  of  sarcoma.  A  his- 
tory of  the  characteristic  prodromes  can  usually  be  obtained  in  glaucoma. 
A  transilluminator  placed  over  the  site  of  a  sarcoma  casts  a  dense  shadow 
on  the  pupil,  but  in  glaucoma  the  pupil  illuminates  perfectly.  Hemor- 
rhagic retinitis  sometimes  is  followed  by  secondary  glaucoma  in  which  the 
history  of  development  closely  resembles  sarcoma.  An  important  diag- 
nostic point  is  the  appearance  of  the  anterior  chamber  which  may  be  of 
normal  or  increased  depth  in  posthemorrhagic  glaucoma. 


Development. 


ACUTE 
GLAUCOMA 


Acute    seizure,    de- 
velops rapidly. 


ACUTE 
IRIDOCYCLITIS. 


Develops  slowb'. 


IRITIS. 


Develops    slowly. 


Pain. 


Violent.  Radiates 
through  all  branches 
of  fifth  nerve.  Con- 
tinuous. 


S  upraorbital 
and  temporal  pain. 
Worse  at  night. 


Eye   tension. 


Always  increased. 


Cornea. 


Anesthetic.     Paren- 
chymatous opacity. 


Frequently    in- 
creased. 


S  upraorbital 
and  temporal  pain. 
Worse  at  night. 


Usually  sensitive. 
Parenchyma  clear. 
Punctate  deposits  on 
posterior  surface  and 
on  lens  are  diagnostic. 


Xormal. 


Clear. 


Anterior  chamber. 


Shallow. 


Usually,   normal   or 
deepened. 


Xormal 


Congested  and  dis- 
colored.    No  nodules. 


Congested   and   dis-        r-  .1        j    j- 

colored.     Mav  contain         ^^"f  ^^^f   ^""^ r.fX 
nodules     {condylo-     ^'^^'^'f'    ^I^Y  contam 


Pupil. 


Dilated, 
immobile. 


oval     and 


Contracted.  May 
be  bound  to  lens  by 
synechia;. 


Contracted.  Irreg- 
ular a  +  t  e  r  dilation 
vvith  atropine.  Syne- 
chi^e. 


GLAUCOMA.  269 

BihlioyrapJiy. 

Baques,  A.   f.  O.  LXVIII,  p.    171,  2,  Oph.  Rev.   Aug.,  1908,  245. 

Donders,  A.  f.  O.  VIII,  2,  1862;     IX,  2,  1863:    B.  d.  o.  G.,  1864. 

Fuclis,   A.  f.  O.,  Bd.  XXX,  :^.  1884,  125.    Text  Book,  Eleventh  Am.,  415. 

Henderson,   T.   O.   S.,   1908,   47. 

Hess.  A.  of  Oph.  Jan,  1912,  XLl,  29. 

Parsons,    Path.   Of   The   Eve,   vol.   Ill,   1047. 

Risley,   Ophthalmologv,   VI,  4,   1910,  572. 

Schiotz,  Tr.  A.  Knapp,  A.  of  O.  XL,  5,  1911.  518. 

Schnabel.   A.   f.   A.,  XXIV.   1892;    Wehi.   m.  Woch..   1900;    Centb.   f.   A.   1908.  143. 

Smith     Priestley.    Glaucoma,    London.    1879,    1881;     Oph.   Rev.    Apl.    1911. 

Von  Hippel,  A.  f.  O.  LI  I,  3,  1901,  496. 

Von   Graefe,   A.    f.   O.   I,   1,    1854;     II,   1,   1855;     1857;     1858;     1862;     1869. 

Goldzieher,   C,   f.  p.   A.   XXVIII,   1904,  257. 

Inoyu,  O.  H.  Rep.,  XVIII,  1910,  24. 


Chapter  XIV. 

INTRAOCULAR  AND  ORBITAL  TUMORS. 

Sarcoma.  The  most  frequent  intraocular  tumor  is  sarcoma  of  the 
choroid.  Statistics  indicate  that  it  occurs  about  once  in  every  1500  cases 
of  eye  disease.  No  age  is  exempt,  but  more  than  half  of  all  cases  occur 
between  the  40th  and  60th  years.  In  common  with  other  progressive  in- 
traocular growths,  sarcoma  passes  through  four  stages  of  development: 

( 1 )  The  preglaucomatous  stage,  in  which  the  eye  is  free  from  pain 
or  irritation.  A  defect  exists  in  the  visual  field  which,  if  central,  will  be 
recognized  by  the  patient.  If  the  tumor  is  far  forward  vision  will  not  be 
disturbed  until  collateral  detachment  of  the  retina  ensues.  In  small  sarco- 
mata the  retina  usually  is  in  contact  with  the  tumor  or  separated  from 
:t  by  only  a  thin  layer  of  fluid  in  which  case  the  color  and  form  of  the 
neoplasm  may  be  seen.  Exceptionally,  however,  a  very  small  sarcoma 
produces  extensive  detachment.  In  all  cases,  as  the  sarcoma  increases  in 
size,  total  detachment  results.  The  usual  duration  of  this  stage  is  from 
six  months  to  a  year;  rarely,  it  continues  for  years  (Fuchs). 

(2)  The  glaucomatous  or  inflammatory  stage,  characterized  by  a 
progressive,  pernicious  glaucoma  which  rapidly  becomes  absolute.  The 
duration  of  the  second  stage  is  shorter  than  that  of  the  first. 

(3)  Stage  of  extrabulbar  extension,  which  enaues  when  the  sarcoma 
breaks  through  the  sclera.  Perforation  usually  occurs  by  extension  along 
blood  vessels  or  along  the  optic  nerve  (Fig.  196),  very  rarely  along  the 
ciliary  nerves.  The  opening  thus  made  in  the  sclera  immediately  relieves 
intraocular  pressure,  and  pain  disappears.  The  tumor,  released  from 
pressure,  grows  with  increased  rapidity.  If  sarcoma  perforates  the  globe 
posteriorly,  it  proliferates  luxuriantly  in  the  orbit  and  causes  exophthal- 
mus.  If  it  emerges  along  an  anterior  ciliary  vessel,  sarcoma  forms  an 
external  granulating  mass,  usually  pigmented. 

(4)  Usually,  the  liver  is  the  first  organ  to  receive  a  metastatic  de- 
posit. It  is  said  that,  as  a  rare  event,  primary  sarcoma  of  the  choroid 
may  send  out  a  metastasis  before  involvement  of  the  sclera.  Reports  of 
such   cases,   however,   suggest   incomplete   microscopic   examination. 

270 


Fig.  192. 


Fig.  193 — Formation  of  Vascular- 
ized Adventitious  Tissue  in  Glauco- 
matous   Excavation. 


Fig.  Wo. 


Fig.  194 — Fibrous  Elevation  Pro- 
jecting from  the  Floor  of  a  Glaucoma- 
tous Excavation  and  Containing  a 
Partially    Obliterated    Blood-vessel. 


Fig.  195 — Buphthalmus.  The  Iris  has 
Remained  in  Contact  with  the  Cornea  at 
the  Ang-le  of  the  Anterior  Chamber.  P, 
pectinate  ligment;    S,  canal  of  Schlemni. 


Fig.  195. 


Fig.  196 — Sarcoma  of  the  Choroid.  Com- 
mencement of  Stage  of  Extra-bulbar  Ex- 
tension,- 


Fig.  196. 


Fig.    197 — Sarcoma    of   the   Choroid,    Pro- 
jecting into  the   Vitreous. 


Fig.  197. 


Fig.  IDS.  Fig.  190. 

Figs.   11)8  and   199 — Sarcoma  of  the   Clioroid   Projecting  into  the   \'itreous. 


Fig.   200 — Flat   Sarcoma   of  the   Choroid. 


Fig.  201. 


Fig.   201 — Endothelioma    of   the    Choroid 

(Alveolar  .Sarcoma). 


INTRAOCULAR    AND    ORBITAL    TUMORS.  271 

In  exceptional  cases  the  glaucomatous  stage  of  sarcoma  is  replaced 
or  followed  by  plastic  iridocyclitis,  atropfiy  of  the  eye  and  apparent  re- 
trocession of  the  growth.  In  some  of  these  cases  the  iridocyclitis  may  be 
merely  coincidental.  In  others  the  sarcoma  undergoes  necrosis  from  de- 
fective nutrition  or  hemorrhage  and  toxins  are  generated  which  excite  in- 
flammation in  the  iris  and  ciliary  processes.  In  all  cases,  the  sarcoma 
progresses  and  ultimately  becomes  actively  malignant. 

The  glaucoma  which  supervenes  upon  sarcoma  and  other  intraocular 
growths  appears  to  be  largely  mechanical,  the  thickened  vitreous  pushing 
forward  the  lens  and  root  of  the  iris,  thereby  closing  the  filtration  angle  in 
the  anterior  chamber.  Contributing  factors  are  obstruction  of  the  filtra- 
tion spaces  by  cellular  elements,  presence  of  albumin  and  fibrin  in  the 
aqueous,  proliferation  of  the  trabeculae  in  the  pectinate  ligament  from  the 
irritating  action  of  toxins  in  the  aqueous  and,  possibly,  congestion  due  to 
pressure  upon   the  vens   vorticosae.* 

In  the  majority  of  cases  sarcoma  of  the  choroid  form.s  a  rounded 
protuberance  situated  on  a  flat,  disciform  base.  This  form  is  determined 
by  environment;  thus,  in  its  early  stage  the  neoplasm  being  compressed 
between  the  sclera  and  lamina  vitrea,  is  low  and  flat.  Soon,  the  lamina 
ruptures  and  the  sarcoma  grows  rapidly  into  the  soft  vitreous  where  it 
assumes  its  characteristic  mushroom  shape,  consisting  of  a  globular  head 
in  the  vitreous,  a  neck  formed  by  constriction  of  the  lamina  vitrea  and  a 
flat  disc  situated  beneath  the  lamina  (Figs.  197-198-199).  Occasionally, 
the  lamina  is  not  perforated  but  yields  uniformly  to  a  slow-growing  sar- 
coma and  a  flat  discoid  tumor  results  (Fig.  200).  A  few  cases  have  been 
reported  in  which  so-called  sarcoma  appeared  as  a  flat,  disseminated  Infil- 
tration of  the  choroid;  histologically,  these  growths  are  endothellomata 
(Fig.  201).  Sarcoma  of  the  choroid,  probably,  always  is  primary.  Cases 
reported  as  metastatic  have  not  been  satisfactorily  established.  The  con- 
ditions necessary  to  establish  the  diagnosis  of  metastatic  sarcoma  of  the 
choroid  are  the  development  of  sarcoma  In  an  eye  known  to  be  previously 
healthy  In  a  person  already  affected  by  sarcoma  elsewhere.  Sarcoma  Is  a 
solitary  growth  and  it  is  extremely  rare  for  a  secondary  deposit  to  form  In 
the  affected  eye,  differing  In  this  respect  from  glioma  of  the  retina,  me- 


*  See  pages   153-4   and  260. 


272  THE    FUNDUS    OCULI. 

tastatic  carcinoma  of  the  choroid  and  conglomerate  tubercle.  I  have  seen 
one  case  of  local  metastasis  in  flat  sarcoma  (endothelioma)  of  the  ciliary 
body. 

A  woman  40  years  of  age  entered  the  clinic  with  no  perception  of  light 
in  the  right  eye,  high  intraocular  pressure  and  cataractous  lens.  The  history 
and  examination  indicated  intraocular  tumor  and  the  eye  was  enucleated. 
Microscopic  examination  showed  the  ciliary  body  entirely  replaced  by  a 
typical  flat  sarcoma  (endothelioma,  sarcoma  carcinomatodes) .  On  the 
optic  nerve  was  a  similar  growth  which  formed  a  globular  protuberance 
in  the  vitreous  and  also  extended  into  the  orbit  (Fig.  201).  These  ciliary 
tumors  always  are  of  extremely  slow  growth,  while  vitreous  and  orbital 
deposits  develop  very  rapidly;  therefore,  the  ciliary  tumor  is  regarded  as 
the  primary  tumor,  particularly,  as  it  corresponded  in  all  respects  to  other 
reported  cases  of  flat  sarcoma.  The  globe  was  sectioned  serially  and  no 
connection  existed  between  the  two  deposits. 

A  few  cases  have  been  reported  in  which  with  sarcoma  in  one  eve, 
a  plastic  iridocyclitis,  supposed  to  be  sympathetic,  has  developed  in  the 
other.  The  assumption  that  these  are  cases  of  sympathetic  ophthalmitis 
does  not  appear  to  be  justified,  inasmuch  as  iridocyclitis  is  a  common  dis- 
ease and  no  more  frequent  among  those  aftected  with  sarcoma  tha.n  among 
others. 

Sarcoma  of  the  choroid,  like  sarcoma  in  other  regions,  consists  of  an 
embryonic  type  of  connective  tissue  cells  thickly  embedded  in  a  scanty, 
intercellular  substance.  The  predominating  type  of  cell  in  a  given  tumor 
may  be  spindle-shaped  or  round.  Frequently,  the  two  types  are  mixed. 
The  occurrence  of  true  giant-celled  sarcoma  in  the  choroid  has  not  been 
established.  Sarcoma  cells  vary  greatly  in  size.  Spindle  celled  tumors 
are  by  far  the  most  common  in  the  choroid.  The  spindle  cells  have  large 
oval  nuclei  and  frequently  their  extremities  bifurcate.  They  are  arranged 
in  closely  packed,  interlacing  bundles  (Fig.  202).  In  round  celled  sar- 
coma the  cells  often  are  irregular  in  shape.  Sarcomata  usually  are  very 
vascular.  The  newly  developed  blood  vessels  consist  of  thin,  endothelial 
lined  tubes  and  channels,  embedded  in  tumor  cells.  As  a  rule  round  celled 
sarcomata  are  more  vascular  than  the  spindle-celled  type.  A  sarcoma 
may  consist  almost  wholly  of  blood  channels  or  tubes  composed  of  cells. 
Such  growths  are  termed  angiosarcomata  and  possess  a  gross  microscopic 
resemblance  to  glioma  of  the  retina  (Fig.  203).  Round-celled  sarcomata 
are  more  rapidly  malignant  than  spindle-celled  tumors. 


Fig.  202  —  Spindle 
celled  Sarcoma  of 
the  Choroid. 


Fig.  202. 


Fig.     203  —  Angio-Sarcoma 
of    the    Choroid. 


Fig.  203. 


Fig.  204. 


.     Fig.  204 — Alveolar  Sarcoma  of  the  Choroid  (endothelioma).    / 


Fig.  205.  ^  Fig.  206.  * 

Fig.  205 — Tumor  of  Choroid  and  Ciliary  Body,  which  on  section  (Fig.  206)  proved 
to  be  a  Perithelioma.  Note  the  Rosette-like  Arrangement  of  the  Cells,  resembling 
Glioma. 


IXIRAOCULAR    AND    ORBMAL    TUMORS.  273 

Alveolar  sarcoma  (endothelioma,  sarcoma  carcinomatodes)  appears 
in  the  uvea  as  the  flat,  diffuse  sarcoma  of  the  choroid  and  as  the  ring  sar- 
coma of  the  ciliary  body  (Fig.  201).  Probably,  these  grovvths  arise  from 
the  endothelium  of  the  lymph  clefts  and  from  the  perivascular  lymph  chan- 
nels. Histologically  they  are  composed  of  large  endothelial  cells  in  a 
distinct  reticulum  (Fig.  204).  These  growths  differ  from  sarcoma  in 
origin,  type  of  cell,  structure,  tendency  to  local  metastasis  and  relatively 
low  malignancy.  They  are  classed  with  sarcoma,  however,  because  both 
develop  from  mesoblastic  tissue. 

A  rare  type  of  angiosarcoma  or  better,  endothelioma,  is  termed  per- 
ithelioma. This  growth  is  distinguished  from  other  endotheliomata  by 
the  type  and  arrangement  of  its  cells.  I  have  seen  but  one  example  of  these 
growths*  (Fig.  2'05).  The  cells  are  of  columnar  or  oval  endothelial  type 
and  possess  abundant  protoplasm  and  rounded  or  elongated  nuclei.  They 
grow  in  a  radiate  manner  around  the  blood  channels  in  immediate  contact 
with  the  endothelial  membrane  (Fig.  206).  Such  vessels,  when  cut  trans- 
versely, appear  as  cellular  rosettes  resembling  those  found  in  glioma  of 
the  retina.  These  tumors  are  supposed  to  develop  from  the  perithelium 
of  the  blood  vessels.  The  perithelium  is  a  single  layer  of  flat  endotheloid 
cells  situated  between  the  adventitia  and  perivascular  lymph  space,  and 
exists  only  in  certain  organs. 

A  convenient  clinical  classification  of  sarcoma  of  the  choroid  as  mel- 
anosarcoma  and  leucosarcoma  is  based  on  the  color  or  degree  of  pigmen- 
tation which  the  tumor  microscopically  presents.  Those  classed  as  leu- 
cosarcoma usually,  if  not  invariably,  contain  some  pigment  granules  visible 
under  the  microscope.  Fuchs  estimates  that  88  per  cent  should  be  classed 
as  melanotic.  The  pigment  occurs  both  in  the  cellular  protoplasm  and  iso- 
lated (Fig.  207).  Much  controversy  exists  as  to  which  layer  of  the 
choroid  is  the  starting  point  of  sarcoma.  Probably,  Shieck  is  correct  in 
his  statement  that  sarcoma  of  the  choroid  is  no  exception  to  the  rule  that 
sarcoma  may  develop  wherever  connective  tissue  normally  exists  and  there- 
fore may  arise  from  the  choriocapillaris  as  well  as  from  the  other  layers. 

The  mortality  from  sarcoma  of  the  choroid  could  be  reduced  if 
instead  of  preserving  enucleated  specimens  for  microscopic  demonstration 
they  were  always  subjected  to  rigorous  microscopic  examination  for  evl- 


*  The  eye  was  microscopically  examined  for  Dr.  Jerome  B.  Thomas. 
18 


274  THE    FUNDUS    OCULI. 

dence  of  extraocular  extension.  The  early  migration  of  tumor  cells  along 
the  optic  nerve  or  the  perforating  vessels  is  invisible  to  the  naked  eye  but 
may  be  discovered  with  the  microscope  and  prompt  removal  of  the  orbital 
contents  may  be  expected  to  effect  a  cure  (Fig.  208).  On  the  other  hand, 
if  left  undisturbed,  recurrence  and  death  are  almost  certain. 

StereogrAxM  74.  Sarcoma  of  the  Choroid  (Leucosarcoma).* 
Right  fundus  oculi  of  a  healthy  farmer,  44  years  of  age.  Left  eye  nor- 
mal. In  the  right  eye  vision  has  gradually  been  disappearing  for  the  past 
six  months.  Obscuration  began  on  the  temporal  side  and  slowly  extended 
like  a  black  curtain  being  drawn  across  the  visual  field.  There  has  been 
no  pain  in  the  eye.  Present  cofidit'wn  :  Externally,  the  eye  appears  healthy. 
Tension  normal.  The  ophthalmoscope  reveals  a  circumscribed,  globular 
tumor  at  the  posterior  pole,  close  to  the  nerve,  on  the  nasal  side.  This 
is  diagnosticated  as  sarcoma  from  its  form,  immobility  on  movement  of 
the  eye  and  light  flesh  color.  Several  large  vessels  cross  its  surface  which, 
from  their  direction  and  method  of  division  are  supposed  to  be  retinal. 
No  adventitious  vessels  are  seen.  A  small,  simple  detachment  of  the  re- 
tina atijoins  the  tumor  below,  recognized  by  a  slight  tremor  of  the  surface 
on  sudden  mov^ement  of  the  eye,  and  also  by  its  color. 

The  eye  was  enucleated  and  microscopic  examination  showed  that  the 
tumor  seen  with  the  ophthalmoscope  was  a  small  leucosarcoma  (Fig.  209). 

Dl'\gnosis.  The  diagnosis  of  sarcoma  of  the  choroid  is  compara- 
tively easy  when,  as  in  the  above  case,  there  is  but  little  collateral  detach- 
ment of  the  retina.  It  becomes  difficult,  however,  when  the  tumor  is  small 
and  the  detachment  total.  In  ev'ery  detachment  the  possible  presence  of 
intraocular  tumor  should  be  considered.  Even  traumatic  detachments  are 
not  beyond  suspicion,  inasmuch  as  slight  injury  suffices  to  produce  detach- 
ment when  a  tumor  exists  in  the  eye.  The  ophthalmoscopic  picture  of  sar- 
comatous detachment  varies.  Where  the  retina  lies  in  contact  with  the 
tumor  or  the  layer  of  fluid  between  tumor  and  retina  is  thin,  the  surface 
will  be  smooth  and  the  vessels  will  not  be  crinkled  as  they  are  in  simple 
detachment.  If,  at  the  same  time,  the  retina  retains  its  transparency 
the  color  and  form  of  the  underlying  tumor  will  be  visible  and  anomalous 
vessels  belonging  to  the  growth  sometimes  are  seen.  Partial  sarcomatous 
detachment  soon  becomes  total;  the  retina  becomes  opaque  and  the  pres- 

*  Patient  was  under  the  care  of  Dr.  David  Webster,  Manhattan  Eye,  Ear  and  Throat 
Hospital. 


Fig.  207 — Melano-Sarcoma  of  the 
Choroid,  Showing  Pigment  in  the 
Cells  and  Isolated. 


Fig.  'Ml 


Fig.  208 — Sarcoma  of  the  Choroid. 
The  Tumor  (S)  has  Invaded  the 
Optic  Nerve  and  Passed  Through  the 
Sclera. 


Fig.  L'US. 


Fig.    209 — Small    Leuco-Sarcoma    at 
the  Posterior  Pole. 


Fig.  209. 


1\1  RAOCILAK    AXD    ORBITAL    TUMORS.  275 

ence  of  intraocular  tumor  can  no  longer  be  determined  with  the  opthal- 
moscope.  At  this  stage  the  diagnosis  is  made  from  the  history  and  by 
aid  of  transillumination.  A  sarcoma  situated  in  the  posterior  hemisphere 
or  below  the  horizontal  meridian  will  be  hidden  by  detachment  earlier 
than  one  anterior  to  the  equator  or  in  the  upper  part  of  the  eye.  Even 
while  the  tumor  is  small,  more  or  less  detachment  of  the  retina  usually 
exists  below  and,  not  infrequently,  an  extensive  detachment  may  be  pro- 
duced by  a  very  small  sarcoma,  in  which  case  the  latter  may  be  overlooked. 
When  the  glaucomatous  stage  ensues  the  detachment  is  pushed  forward 
against  the  lens,  the  retinal  folds  are  crowded  together  and  the  broad 
surface  furrows  of  simple  detachment  are  transformed  into  narrow  slits. 
Simple  detachment  usually  occurs  in  myopic  eyes,  spontaneously  or  from 
slight  injury.  In  any  eye,  detachment  may  be  produced  by  traumatism, 
albuminuria,  subretinal  exudation  or  hemorrhage,  contraction  of  retinal 
cicatrices,  loss  of  support  from  diminution  in  the  size  of  the  vitreous  and 
contraction  of  vitreous  exudates.  These  causes  must  be  considered  and 
excluded  before  diagnosticating  a  detachment  as  due  to  sarcoma  of  the 
choroid.  Eye  tension  is  not  increased  and  may  be  diminished  in  simple 
detachment;  it  is  elevated  or  at  least  normal  in  sarcomatous  detachment. 
Sarcoma  may  be  attended  by  discomfort,  but  simple  detachment  is  painless. 
In  simple  detachment  the  retina  may  become  temporarily  replaced  and  a 
corresponding  improvement  in  vision  will  occur,  but  sarcomatous  detach- 
ment grows  progressively  worse.  In  simple  detachment  the  loss  of  vision 
is  rapid;  in  sarcoma  gradual  loss  of  vision  usually  precedes  total  detach- 
nVent.  Floating  bodies  In  the  vitreous  are  more  common  in  myopic  than 
in  sarcomatous  detachment.  Detachment  due  to  subretinal  hemorrhage 
usually  is  accompanied  by  extensive  bleeding  in  other  parts  of  the  retina. 
The  picture  of  subhyaloid  hemorrhage  is  too  distinctive  to  be  mistaken 
for  tumor  (Stergm.  33). 

Hemorrhagic  retinitis  from  venous  thrombosis  or  other  causes  is 
not  infrequently  followed  by  inflammatory  glaucoma.  As  in  sarcoma  of 
the  choroid,  the  glaucoma  is  unilateral  and  preceded  by  painless  loss  of 
vision.  The  two  conditions  differ,  however,  in  the  following  respects;  in 
sarcoma  vision  usually  disappears  slowly  and  frequently  the  patient  de- 
scribes the  gradual  advance  of  darkness  from  one  side,  while  in  retinal 
hemorrhage  vision  is  lost  rapidly.  In  glaucoma  due  to  sarcoma  the 
anterior  chamber  is  shallow,  but  in  the  early  stage  of  glaucoma  secondary 


276  THE    FUNDUS    UCULl. 

to  retinal  hemorrhage  the  anterior  chamber  usually  is  deep  (see  pages 
151-2,  154). 

Sarcoma  usually  blocks  one  or  more  of  the  vorticose  veins  which 
empty  the  choroid,  and  a  collateral  flow  is  established  through  the  anterior 
ciliary  veins  of  the  same  side.  Therefore,  in  a  doubtful  case,  one  or  more 
greatly  dilated  anterior  ciliary  veins,  limited  to  one  side  of  the  eye,  are 
evidence  in  favor  of  sarcoma. 

The  form  of  the  globe  seldom  is  altered  by  sarcoma  of  the  choroid 
until  perforation  is  imminent,  differing  in  this  respect  from  conglomerate 
tubercle  which  tends  to  soften  the  overlying  sclera,  causing  staphyloma- 
tous  bulging.  In  one  exceptional  case  which  I  microscopically  examined,* 
a  large  equatorial  staphyloma  existed  over  a  sarcoma  of  the  choroid. 
The  cavity  of  the  staphyloma  was  filled  with  disintegrated  blood.  The 
sarcoma  had  not  invaded  either  the  sclera  or  staphyloma,  and  the  latter 
was  produced  by  hemorrhage  from  either  a  vorticose  vein  or  from  the 
sarcoma    (Fig.  210). 

Primary  glaucoma  almost  always  affects  both  eyes,  though  the  dis- 
ease may  be  incipient  in  one  eye  and  advanced  in  the  other;  consequently, 
unilateral  glaucoma  should  always  suggest  the  presence  of  sarcoma. 
Glaucoma  is  attended  by  remissions  and  is  more  or  less  amenable  to  treat- 
ment, but  in  sarcomatous  glaucoma  the  pain  seldom  remits  until  after 
perforation  of  the  globe.  Iridectomy,  sclerotomy,  etc.,  which  relieve 
glaucoma,  hasten  the  advance  of  sarcoma. 

Sarcoma  should  be  differentiated  from  metastatic  carcinoma  of  the 
choroid,  for  the  reason  that  sarcoma  demands  immediate  enucleation, 
while  in  carcinoma  of  the  choroid  the  fatal  termination  is  precipitated  by 
surgical  interference,  the  average  duration  of  life  after  enucleation  being 
less  than  fiv^e  weeks,  although  at  the  time  the  patient  may  appear  in 
good  health. t  Therefore,  in  all  cases  of  intraocular  growth,  search 
should  be  made  for  tumor  elsewhere  in  the  body,  especially  in  the  breast, 
and  if  a  carcinoma  is  discovered  the  eye  should  not  be  enucleated  except  for 
relief  of  pain.  Metastatic  carcinoma  of  the  choroid  appears  as  a  flat  de- 
posit at  the  posterior  pole  (Fig.  211),  but  when  far  advanced  it  may, 
like  sarcoma,  form  a  rounded  protuberance  in  the  vitreous.  I  examined 
microscopically  one  such  case  for  Dr.  P.  C.  Jameson.     A  woman,  aged  38 

*  From  the  service  of  Dr.  J.   Scott  Wood.  Brooklyn   Eye  and  Ear  Hospital, 
t  Oatman,  Am.  Jour.  Aled.  Sci.  :\Iarch  1903. 


Fig.  210. 

Fig.  210 — Sarcoma  of  the  Choroid  in  Connection  with  a  Large  Equatorial  Staphy- 
loma, the  latter  caused  In-  Hemorrhage  and  filled  with  Disintegrated  Blood. 


Fig.  211.  Fig.  212. 

Fig.  211 — Metastatic  Carcinoma  of  the  Choroid,  forming  a  Flat  Tumor  at  the 
Posterior   Pole. 

Fig.  212 — Metastatic  Carcinoma  of  Choroid.  There  are  two  Deposits;  one 
rounded  and  large  at  the  Posterior  Pole;    the  other  flat,  in  the  Ciliary   Region. 


INTRAOCULAR    AND    ORBITAL    TUMORS. 


277 


years,  with  carcinoma  of  the  uterus,  suffered  from  a  metastatic  carcinoma- 
tous deposit  in  the  left  eye.*  When  first  examined  with  the  ophthalmo- 
scope two  deposits  were  seen:  one,  a  large  rounded  tumor  springing  from 
the  posterior  pole,  and  a  second  mass  in  the  ciliary  region  (Fig.  212). 


Sarcoma  of  the  Choroid. 

Always  primary  in  the  choroid. 
May  develop  at  any  point. 

Forms  a  rounded  protuberance 
in  the  vitreous.  Flat  sarcoma  (en- 
dothelioma)   is  extremely  rare. 


Usually  exists  for  many  months 
before  destroying  vision. 

Detachment  occurs  late  when 
tumor  is  centrally  located  (Grif- 
fiths). 

The  pain  appears  to  be  due 
solely  to  high  eye  tension. 

If  tumor  is  limited  to  choroid 
tension  is  not  diminshed. 

Only  one  eye  affected. 


Metastatic  Carcinoma  of  the 
Choroid 

Always  secondary  in  the  choroid. 

Situated  in  posterior  polar  zone; 
usually  on  the  temporal  side  of 
nerve. 

Typical  appearance  is  a  flat,  oval 
deposit  with  a  central  elevation  of 
I  or  2  mm.,  gradually  sloping  into 
the  surrounding  fundus.  Its  color 
is  a  dirty  yellow  with  scattered 
pigment   spots. 

Vision  is  destroyed  in  from  two 
to  eight  weeks  after  appearance 
of  first  eye  symptoms. 

Loss  of  vision  is  due  to  early 
and  extensiv'C  detachment  of  re- 
tina. 

Eye  may  be  very  painful  with 
normal   tension. 

Tension  is  reported  as  increased 
in  one-third,  normal  or  diminished 
(  ?)  in  two-thirds  of  the  cases. 

In  one-third  of  all  cases  both 
eyes  contain  a  carcinomatous  de- 
posit. 


*  The  only  recorded  case  of  primary  carcinoma  of  the  uterus  with  metastasis  in  the 
choroid. 


278  the  fundus  oculi. 

Stereogram  75.  Sarcoma  of  the  Choroid  with  Total  Detach- 
ment OF  the  Retina.  Melanosarcoma.  Right  eyeground  of  a  man  aged 
47  years.  Five  months  ago  he  first  noticed  a  cloud  in  the  upper  field  of  this 
eye,  which  very  gradually  increased  in  size.  Three  weeks  ago  the  eye  rap- 
idly became  blind.  Present  condition.  Right  eye:  The  ciliary  region  is  not 
reddened,  but  below  a  single  dilated  anterior  ciliary  vein  appears  on  the 
sclera.  The  media  are  clear.  Anterior  chamber  appears  of  normal  depth. 
Eye  tension,  by  digital  palpation,  is  normal.  Within  the  past  day  or  two 
he  has  experienced  a  sense  of  discomfort  in  the  eye.  Vision  is  reduced  to 
perception  of  light  in  the  lower  field  and  an  absolute  scotoma  exists  above. 
OpJitJidlnioscopc.  The  retina  is  completely  detached.  The  detachment  is 
recognized  as  such  by  its  folded,  wavy  surface,  grey  "  shot-silk  "  color, 
regularly  distributed  and  darkened  retinal  vessels  and  a  trembling  motion 
imparted  by  movement  of  the  eye.  The  top  of  a  dark  brown  nodular 
tumefaction  Vv'hich  does  not  tremble  when  the  eye  is  moved  is  seen  in  front 
of  and  below  the  detachment.  Transillumination  of  the  eye  from  a  point 
far  back  and  below,  casts  a  shadow  on  the  pupil.  From  other  regions  of 
the  eye  the  pupil  illuminates  perfectly.  The  diagnosis  is  melanosarcoma 
of  the  choroid. 

The  history  of  this  case  indicates  sarcoma.  Development  of  the 
neoplasm  was  attended  by  slow,  painless  loss  of  vision.  The  abrupt  blind- 
ness which  ensued  marks  the  time  when  detachment  occurred. 

1  he  eye  was  enucleated  and  microscopic  examination  showed  the 
tumor  to  be  a  well  pigmented  sarcoma  of  the  choroid   (Fig.   198). 

Bibliography. 

Fuchs,  d.  Sarc.  d.  Uvealtract.  W'ien,  1882.     Fuchs  &  Wintersteiner,  B.  d.  o.  G.   1900. 

Text  Book. 
Schieck.   A.  f.  O..  XLV,  2.  1898;    XLVITI.  w.  1899. 

Glioma  of  the  Retina.  Glioma  of  the  retina  is  a  malignant  growth 
occurring  only  in  infants  and  young  children.  It  rarely,  if  ever,  develops 
after  the  sixth  year;  even  this  is  a  late  age  for  the  first  appearance  of 
glioma  and  such  cases  require  to  be  substantiated  by  authentic  micro- 
scopic examination.  Most  gliomata  are  congenital,  but  owing  to  their 
slow  progress  they  escape  early  observation.  Usually,  however,  discovery 
occurs  before  the  third  year.  Like  sarcoma  of  the  choroid,  glioma  of 
the  retina  passes  through  four  stages: 

(1)    Preglaucomatous  stage,   in  which  it  may  be  difl^cult  or  impos- 
sible to  diagnosticate  the  condition; 


INTRAOCULAR    AND    ORBITAL    TUMORS.  279 

(2)  Glaucomatous  or  inflammatory  stage  which  resembles  the  glau- 
comatous stage  of  sarcoma,  except  that  in  young  children  the  sclera  is 
more  distensible  than  in  adults  and  in  glioma  staphylomatous  bulgings 
precede  perforation; 

(3)  Stage  of  e.xtrahular  extension.  Perforation  is  followed  by 
lowered  eye  tension  and  relief  from  pain.  If  the  opening  is  posterior, 
the  neoplasm  flourishes  in  the  orbit  and  produces  exophthalmus;  if  an- 
terior, a  granulating  mass  develops  which  has  been  termed  fungus 
hematodes. 

(4)  Stage  of  metastases  and  death.  Metastases  occur  early 
and  show  a  preference  for  the  lymphatic  glands,  including  the  preauricular, 
for  the  brain  and  for  the  cranial  bones,  especially  the  facial. 

Glioma  advances  slowly  by  deposit  of  neighboring  metastases  and 
its  course  covers  a  period  of  several  years,  the  victim  finally  dying  from 
exhaustion  or  from  metastatic  deposits  in  vital  parts,  particularly  in  the 
brain.  Death  is  inevitable  unless  the  eye  is  enucleated  before  the  stage 
of  extraorbital  extension.  If  the  growth  does  not  recur  within  three  years 
after  removal,  the  case  may  be  regarded  as  cured.  Glioma  in  the  second 
eye  appears  to  be  an  independent  growth  and  not  a  metastasis  or  ex- 
tension from  the  first  eye;  therefore,  in  bilateral  glioma  a  cure  may  fol- 
low double  enucleation.  An  eye  enucleated  for  glioma  should  be  micro- 
scopically examined  in  serial  sections  and,  if  tumor  elements  have  passed 
into  or  beyond  the  sclera,  the  orbital  contents  should  immediately  be  re- 
moved. Recurrence  frequently  is  due  to  neglect  of  this  precautionary 
measure.  Infants  born  with  manifest  glioma  frequently  are  affected  in 
both  eyes,  while  glioma  which  develops  after  birth  is  unilateral  in  about 
90  per  cent,  of  cases.  When  glioma  develops  in  the  second  eye  its  evo- 
lution may  be  ophthalmoscopically  observed.  In  the  earliest  state  glioma 
may  appear  as  several  light  colored  spots  which  soon  become  elevated 
and  develop  an  independent  vascular  supply.  Usually,  the  first  svmptom 
of  glioma  to  attract  attention  is  a  peculiar  yellowish  or  pinkish  white  light- 
reflex  that  occasionally  flashes  through  the  pupil  as  from  the  eyes  of  cer- 
tain animals;  hence  the  condition  has  been  known  as  amaurotic  cat's  eye. 
This  pupillary  reflex  is  not  pathognomonic  of  glioma  but  occurs  also  in 
cases  of  detached  retina  saturated  with  inflammatory  products,  the  so- 
called  pseudoglioma. 

Stereogram  76.  Glioma  of  the  Retina.  Right  fundus  ocull  of 
a  boy,  aged  2  years.  Apparently  in  good  health.  Family  history  negative. 
Has  had  no  eruptive  fever  or  severe  illness.     The  parents  recently  ob- 


280  THE    FUNDUS    OCULI. 

served  a  peculiar  appearance  of  the  right  eye  consisting  of  a  whitish 
reflex  from  the  pupil.  Eye  tension  normal.  Media  clear.  No  iritic  ad- 
hesions. The  pupil  responds  promptly  to  direct  action  of  light.  By  focal 
illumination  a  deep,  pinkish  white  reflex  is  obtained  through  the  pupil. 
With  the  ophthalmoscope  the  lower  portion  of  the  fundus  is  seen  to  be 
greatly  elevated.  On  the  nasal  side  below  is  a  pinkish  white  tumefaction, 
covered  with  blood  vessels  which  branch  in  an  erratic  manner  and  pursue 
anomalous  courses.  This  mass  has  a  firm  appearance  and  does  not  tremble 
on  movement  of  the  eye,  in  fact,  it  presents  the  ophthalmoscopic  picture 
of  a  neoplasm.  On  the  temporal  side  anci  below  the  tumor  are  large, 
silver  grey,  bladder-like  elevations,  over  which  the  retinal  vessels  pursue 
a  normal  course.  This  region  trembles  on  suciden  movement  of  the  eye 
and,  evidently,  is  a  simple  detachment  of  the  retina.  The  pupil  is  not 
darkened  on  transillumination.  The  diagnosis  is  glioma  with  collateral 
detachment. 

Enucleation  was  performed  and  microscopic  examination  showed  the 
tumor  to  be  a  typical  glioma  retiucC  (Fig.  213).  No  extrabulbar  exten- 
sions of  the  growth  were  discovered.  Two  years  after  enucleation  there 
had  been  no  recurrence. 

Morbid  Anatomy.  In  glioma  secondary  deposits  occur  throughout 
the  eye,  a  condition  extremely  rare  in  sarcoma.  As  a  rule,  glioma  grows 
outward  into  the  subretinal  space  (glioma  exophytum)  ;  less  fre':iuently 
inward  into  the  vitreous  (glioma  endophytum).  The  former  type  pro- 
duces greater  detachment  of  the  retina  than  the  latter.  The  optic  nerve 
is  early  invaded  by  the  tumor  and  converted  into  gliomatous  tissue.  The 
choroid  is  invaded  both  by  metastasis  and  direct  extension  from  the  retina. 
Perforation  usually  occurs  along  the  vessels  which  pass  through  the  sclera 
and  also  along  the  optic  nerve.  Fresh  glioma  is  pinkish  white  or  light 
yellow  in  color  and  of  soft  consistence.  From  its  gross  resemblance  to 
brain  tissue  it  has  been  called  medullary  cancer.  Retinal  glioma  is  con- 
structed of  convoluted  blood  vessels  the  walls  of  which  are  composed  of 
thickly  packed  tumor  cells  (Fig.  214).  These  cell-covered  blood  vessels 
are  separated  from  each  other  by  wide  areas  filled  with  dead  cells  which 
'have  perished  from  malnutrition.  In  the  early  stage  of  development 
glioma  cells  are  arranged  In  radiate  form  around  short,  capillary  tubules 
which  frequently  contain  blood.  Transverse  microscopic  sections  of  these 
tubules  form  the  rosettes  which,  when  present,  are  diagnostic  of  glioma 
(Fig.  215).  In  the  later  stages  this  peculiar  structure  is  lost  and  the 
tumor  presents  the  appearance  of  angiosarcoma.     Even  microscopically. 


Fig.      21:;  — GKoma      uf      the 
Retina. 


Fig.  214.  Fig.  215. 

Fig.  214— Section  of  Retinal  Glioma,  showing  the  Convoluted  Blood  Vessels,  the 
Walls  of  which  are  Filled  with  Tumor  Cells. 

Fig.  215 — The  same,  showing  the  Tumor  Cells  arranged  like  Rosettes  around  the 
Walls  of  the  Capillary  Tubules. 


INTRAOCULAR    AND    ORBITAL    TUMORS.  281 

pseudoglioma  may  present  a  resemblance  to  glioma  sufficient  to  deceiv^e 
the  Inexperienced.  For  example,  I  have  examined  a  case  of  metastatic 
retinitis  following  epidemic  cerebrospinal  meningitis,  in  which  the  massed 
retinal  tissue  was  thrown  into  folds  which  formed  rosettes  that  might  be 
mistaken  for  those  of  glioma.  The  variety  of  cells  found  in  glioma  of 
the  retina  indicates  that  some  of  them  at  least  are  common  to  all  forms 
of  chronic  proliferative  retinitis  and  have  appeared  in  response  to  an 
irritant.  The  tumor  cells  are  embryonic  in  type;  they  are  of  small  size, 
round  or  cylindric  in  form  and  possess  a  large  nucleus  and  scanty  proto- 
plasm. Ganglion  and  neuroglia  spider  cells  can  usually  be  demonstrated 
(Pig.  216).  Glioma  is  strictly  a  nonpigmented  tumor.  If  pigment  is 
present  it  is  either  hematogenous  or  displaced  from  the  retinal  epithe- 
lium. The  intercellular  substance  is  scanty  and  consists  largely  of  cell 
processes.  I  he  development  of  nutritive  blood  vessels  in  glioma  does  not 
keep  pace  with  cellular  proliferation;  consequently,  early  and  extensive 
necrosis  and  degeneration  occur.  The  m.ost  common  change  is  fatty  de- 
generation followed  by  calcification.  Less  frequently  hyaloid  changes 
ensue.  The  metastases  of  glioma  often  histologically  resemble  small 
round-celled  sarcoma.  Glioma  is  said  to  arise  from  the  nuclear  layers  of 
the  retina.  The  name  is  based  upon  its  supposed  origin  from  the  retinal 
neuroglia  (Virchow).  Other  theories  of  origin  are:  (a)  misplaced  neu- 
roglia and  ganglion  cells  (Greeff)  ;  (b)  misplaced  rods  and  cones  (Win- 
tersteiner)  ;  embryonic  retinal  cells  (Treacher  Collins).  The  close  struc- 
tural resemblance  of  glioma  to  perithelioma  (page  280)  suggests  a  com- 
mon vascular  origin. 

Cryptoglionl\.  Under  the  name  of  cryptoglioma  Schobl  has  de- 
scribed rare  cases  in  which,  although  glioma  is  present,  its  symptoms  are 
so  obscured  that  a  true  diagnosis  cannot  be  made.  In  these  cases  glioma 
develops  in  an  eye  but  usually  escapes  observation;  next,  iridocyclitis  de- 
velops, the  globe  softens  and  the  fundus  is  hidden  by  exudates,  cataract  or 
detachment  of  the  retina.  At  this  period  there  is  nothing  except  the  age 
of  the  patient  to  suggest  glioma.  Later  the  glioma  becomes  active  and 
progresses  to  a  fatal  termination.  The  cryptic  character  of  these  cases 
may  be  ascribed  either  to  posterior  perforation  of  the  globe  by  the  glioma, 
or  to  an  iridocyclitis  excited  by  the  products  of  tumor  necrosis.  In  another 
rare  class  of  cases,  glioma  develops  and  then  recedes  without  intercurrent 
iridocyclitis  or  softening  of  the  eyeball.  In  course  of  time,  however,  the 
neoplasm  reappears.  Glioma  possesses  a  great  tendency  to  degenerate, 
and  in  these  cases  of  recession  it  is  probable  that  a  large  part  of  the  tumor 


282  THE    FUNDUS    OCULI. 

dies  and  is  removed  by  absorption.  Cases  of  cryptoglioma  are  so  rare 
as  to  exert  but  little  influence  upon  differential  diagnosis.  Parsons  found 
15  alleged  cases  in  literature.  In  but  six  of  these  was  the  microscopic 
examination  at  all  satisfactory.  Parsons  says  of  all  that  "  the  gliomatous 
nature  of  cells  in  these  shrunken  eyes  is  not  placed  absolutely  beyond 
cavil."  Cases  of  supposed  cryptoglioma  require  to  be  carefully  differen- 
tiated from  tuberculoma. 

PsEUDOGLlOMA.  The  name  "  pseudoglioma  "  is  applied  to  any  non- 
malignant,  intraocular  formation  in  children  which  bears  a  clinical  re- 
semblance to  glioma  of  the  retina.  Pseudoglioma  usually  is  an  inflamma- 
tory formation  situated  in  either  the  retina,  the  choroid  or  both;  less  fre- 
quently in  the  vitreous.  The  majority  of  cases  occur  during  the  course 
of  an  infectious  disease,  particularly  epidemic  cerebrospinal  meningitis, 
and  are  caused  by  bacterial  metastasis.  If  a  metastasis  of  pyogenic  or- 
ganisms lodges  in  the  eye  it  is  followed  by  suppuration  of  the  globe 
(panophthalmitis).  Pseudoglioma,  however,  follows  a  metastasis  of 
nonpyogenic  bacteria  which  excites  plastic  exudation  rather  than  pus  for- 
mation. Inasmuch  as  any  of  several  intraocular  parts  may  be  the  seat  of  a 
metastasis,  the  inflammation  which  results  may  be  designated  metastatic 
endophthalmitis.  When  both  eyes  are  attacked  the  blindness  which  results 
immediately  attracts  attention,  but  a  unilateral  metastatic  endophthalmitis 
occurring  in  the  course  of  an  acute  fever  usually  escapes  observation 
especially  as  the  eye  seldom  exhibits  external  irritation,  then  or  afterward. 
Intraocular  infection  occurs  most  frequently  through  penetrating  wounds 
which  either  destroy  the  eye  or  leave  the  lens  and  cornea  opaque.  Occa- 
sionally, however,  a  wound  may  leave  the  media  transparent  and  also  re- 
sult in  formation  of  cicatricial  tissue  which,  ophthalmoscopically,  resembles 
a  malignant  neoplasm.  In  these  traumatic  cases  the  exudati\'e  process 
usually  is  more  circumscribed  than  in  metastatic  endophthalmitis  and  it 
may  be  associated  with  simple  detachment  of  the  retina.  A  persistent 
fetal  fibrovascular  lens  sheath  has  been  mistaken  for  glioma.  This  mal- 
formation usually  is  associated  with  persistent  hyaloid  artery  and  unde- 
veloped lens  and  capsule.  It  presents  a  shining  white  surface  covered 
with  blood  vessels. 

Various  inflammatory  conditions  may  result  in  a  pseudoglioma.  The 
most  frequent  is  metastatic  endophthalmitis  with  detachment  of  the  ret- 
ina. In  these  cases  the  posterior  portion  of  the  retina  is  gathered  into 
a  cord  which  extends  from  the  optic  nerve  to  the  anterior  part  of  the 
vitreous  cavity  where  it  expands  to  its  attachment  around  the  ora  serrata, 


Fig.  216 — Glioma  of  the  Retina 
Showing  Ganglion  and  Neuroglia 
Spider  Cells. 


F.^.   lilO. 


Fig.    217 — Umbrella-like    Detachment    of   the    Retina   fol 
lowing  Metastatic   Endophthalmitis. 


Fig.  217. 


Fig.  218— The  same  as  217.  The 
Choroid  is  Not  Detached  in  Either 
Specimen,  best  shown  in   Fig.  218. 


Fig.  218. 


INTRAOCULAR    AND    ORBITAL    TUMORS.  283 

forming  the  so-called  umbrella-form  detachment  (Figs.  217-218).  In 
metastatic  choroiditis  the  retina  may  not  detach,  but  remain  glued  to  the 
thickened  choroid.  Exudation  from  the  ciliary  body,  especially  after 
injury,  may  form  a  tumor-like  mass  in  the  v^Itreous  which  becomes  encysted 
and  may  develop  blood  vessels.  The  majority  of  pseudogllomas  which  I 
have  microscopically  examined  were  cases  of  metastatic  retinitis;  less  fre- 
quently metastatic  choroiditis  was  found.  In  metastatic  retliiltis  the  con- 
traction of  cicatricial  bands  may  drag  the  ciliary  body  from  its  bed  even 
though  there  has  been  no  cyclltls.     If  cyclitis  develops,  the  eye  softens. 

As  the  result  of  disease  or  injury,  exudate  from  the  posterior  por- 
tion of  the  ciliary  body  may  be  poured  out  into  the  post-lental  region  and 
organize  Into  a  permanent  membrane  on  the  anterior  surface  of  the 
vitreous. 

Stereogram  77.  Pseudoglioma  (Metastatic  Endophthalmitis). 
Left  eyeground  of  a  boy,  seven  years  of  age.  His  only  severe  sickness 
was  from  diphtheria,  five  years  ago.  Right  eye,  normal.  His  parents 
recently  discovered  that  his  left  eye  is  blind.  Since  birth  the  left  iris  has 
been  lighter  In  color  than  the  right.  No  perception  of  light.  The  pupil 
reacts  to  a  direct  strong  light,  but  is  abnormally  sluggish  by  weak  illumi- 
nation. Consensual  pupillary  reaction  is  normal.  The  ophthalmoscope 
shows  the  entire  fundus  to  be  unevenly  elevated.  Bluish  white,  nodular 
formations  cover  the  disc  and  also  the  Inferior  and  nasal  portions  of  the 
eyeground.  The  upper  outer  quadrant  of  the  fundus  appears  as  an  im- 
movable, flat,  pinkish  white  membrane.  The  entire  surface  of  the  eye- 
ground  Is  covered  with  blood  vessels  some  of  which  are  newly  formed 
but  the  majority,  from  their  method  of  distribution,  are  recognized  as 
retinal.  Many  of  the  retinal  vessels  pierce  the  nodules,  a  peculiarity  which 
suggests  that  the  latter  are  composed  of  Inflammatory  exudate  which  has 
covered  the  vessels.  The  flat  area  extending  upward  and  outward  is  sup- 
posed to  be  detached  retina  containing  less  exudate  than  other  regions. 
The  narrow,  pointed,  white  formation  In  the  lower  border  of  the  flat  area 
is  regarded  as  a  band  of  newly  formed  connective  tissue. 

The  diagnosis  Is  metastatic  endophthalmitis.  Probably,  the  choroid 
was  the  seat  of  metastasis  and  the  retina  was  fastened  to  the  choroid  bv 
inflammatory  exudates. 

Diagnosis.  In  the  first  stage,  glioma  presents  no  positive  diagnostic 
sign;  consequently,  Its  recognition  often  Is  dlfl'icult  or  even  Impossible. 
Differentiation  usually  lies  between  glioma  and  a  detached  retina  filled 
with  Inflammatory  products   (pseudoglioma).     In  these  cases  an  accurate 


284  THE    FUNDUS    OCULI. 

decision  is  demanded.  An  eye  containing  a  pseudoglioma  is  blind,  but  it 
is  innocuous  and  may  present  a  normal  external  appearance.  Removal 
of  such  an  eye  disfigures  the  child  and  prevents  proper  development  of 
the  corresponding  side  of  the  face.  On  the  other  hand,  failure  to  enucleate 
a  gliomatous  eye  results  in  the  patient's  death.  The  history  is  im- 
portant. In  certain  families  several  children  perish  from  glioma  retinae. 
Many  solitary  cases,  however,  occur.  History  of  an  infectious  fever  can 
usually  be  obtained  in  pseudoglioma.  The  presence  of  glioma  is  dis- 
covered before  the  third  year  in  two-thirds  of  all  cases,  and  the  prob- 
abilities are  strongly  against  its  development  beyond  the  age  of  five 
years.  As  our  knowledge  of  glioma  advances,  late  cases  become  more 
infrequent.  Iritic  adhesions  anci  deposits  on  the  lens  are  evidence  in 
favor  of  pseudoglioma.^  Glioma  nodules  may  develop  on  the  iris  in 
advanced  cases,  but  are  rare  in  the  early  stages  when  the  diagnosis  is 
in  doubt.  Glioma  usually  presents  a  clear  anterior  chamber,  although 
hyphema  and  deposits  of  tumor  debris  somewhat  resembling  hypopyon 
have  been  reported.  True  hypopyon  of  a  decided  yellow  color  may 
form  in  metastatic  endophthalmitis.  Previous  to  the  glaucomatous 
stage  the  pupillary  reactions  possess  considerable  ciiagnostic  value. 
Thus,  in  glioma  only  a  limited  area  of  retina  is  clestroyed  and  enough 
percipient  elements  remain  to  cause  a  prompt  response  of  the  pupil  to 
light.  Therefore,  in  glioma  the  pupil  reacts  well  to  direct  light.  In  met- 
astatic endophthalmitis,  on  the  contrary,  the  entire  retina  may  be  con- 
verted into  a  fibrous  membrane  which  retains  few  or  no  sensitive  elements 
and  the  pupil  will  respond  sluggishly  or  not  at  all  to  direct  action  of  light, 
while  consensual  action  is  normal.  Often,  however,  retinal  tissue  suffi- 
cient to  produce  pupillary  response  persists  for  a  long  time.  Conse- 
quently, in  pseudoglioma  the  pupil  may  or  may  not  react  to  light,  but  an 
inactive  pupil  is  evidence  against  the  presence  of  glioma.  As  a  rule,  eye 
tension  is  normal  or  lowered  in  metastatic  ophthalmitis  anci  normal  or 
heightened  in  glioma.  In  old  metastatic  endophthalmitis  tension  may 
rise  from  sudden  recrudescence  of  the  inflammatory  process  (Fig.  219) 
or  from  occlusion  of  the  pupil  in  which  cases  the  cause  of  the  glaucoma  is 
obvious.  In  nearly  all  doubtful  cases,  however,  if  tension  is  increased  there 
is  glioma  and  if  lowered  there  is  pseudoglioma.  In  soft  eyes  the  pos- 
sible existence  of  cryptoglioma  should  be  borne  in  mind  and  the  child 
kept  under  observation.  The  pinkish-white  color  of  glioma  may  be  du- 
plicated in  certain  parts  of  an  old  inflammatory  exudate,  but  other  parts 
will  exhibit  either  strong  yellow,  shining  white  or  bluish  colors  that  do 


Fig.  219 — Metastatic  Endophthal- 
mitis. Tension  was  Increased  in  this 
case  on  account  of  a  Recrudescence 
of   the  Infiammatory  Process. 


Fig.  210. 


Fig.  220 — Massive,  Circumscribed  Exu- 
dation, due  to  Injury  and  Ophthalmri- 
scopically  Simulating  Tumor. 


Fig.  231 — Post-lental  Memljranous  Exu- 
date (shown  at  P.  in  lower  left  of  the 
f-gure). 


Fig.  221. 


INTRAOCULAR    AND    ORBITAL    TUMORS.  285 

not  belong  to  glioma.  The  surface  of  glioma  often  presents  discoloration 
due  to  hemorrhage,  necrosis  or  calcification  quite  different  in  appearance 
from  the  bands  and  sheets  of  connective  tissue  seen  in  some  cases  of  or- 
ganized exudate.  Adventitious  blood  vessels  are  nearly  constant  in  glioma 
and  not  uncommon  in  pseudoglioma.  x'\bsence  of  all  vessels  on  a  tumor 
points  to  pseudoglioma.  Infiammatory  new  formations  may  ensheath 
the  vessels,  an  appearance  which  I  have  never  seen  in  glioma.  The  ap- 
pearance of  the  detached  retina  may  throw  some  light  on  the  nature  of 
an  intraocular  tumor.  Thus,  in  metastatic  endophthalmitis  the  retina 
usually  is  totally  detached  and  saturated  with  inflammatory  material  or 
converted  into  fibrous  tissue  which  renders  the  membrane  rigid  and  im- 
movable on  movement  of  the  eye.  In  glioma,  however,  there  may  be  ex- 
tensive collateral  simple  detachment  which  irembles  on  movement;  there- 
fore, in  a  doubtful  case  the  presence  of  a  simple  detachment  with  a  trem- 
ulous surface  points  to  the  presence  of  glioma. 

Massive,  intraocular  exudation  following  injury  may  present  a  puz- 
zling ophthalmoscopic  picture  consisting  of  a  circumscribed  exudation 
(Fig.  220).  In  traumatic  cases,  however,  evidence  of  an  injury  can  usu- 
ally be   obtained. 

Post-lental  cyclltic  membranes  or  exudates  are  best  seen  by  focal  il- 
lumination. I  hey  present  a  smooth,  bluish-white  surface  which  bears  little 
resemblance  to  glioma    (Fig.  221). 

After  metastatic  choroiditis  or  uveitis,  the  vitreous  cavity  may  be 
filled  with  a  purulent  fluid  and  the  post-lental  surface  appears  uniformly 
yellow.  In  advanced  cases  of  glioma  the  preauricular  glands  enlarge  and 
nodules  may  form  on  the  head  and  facial  bones. 

Transillumination  is  less  satisfactory  in  the  diagnosis  of  glioma  than 
in  sarcoma.  As  a  rule,  pupillary  illumination  is  but  slightly  dimmed  by 
glioma.  This  is  due  to  the  following  conditions:  (a)  glioma  is  a  retinal 
growth  and  more  or  less  space  exists  between  tumor  and  sclera  through 
which  light  reaches  the  pupil;  (b)  glioma  contains  no  pigment;  (c)  the 
vascular  interspaces  are  filled  largely  by  degenerated  tissue  which  does 
not  obstruct  light.  A  glioma  mav  obstruct  light  if  it  has  extensively  in- 
vaded the  choroid  and  especially  if  it  also  contains  a  large  blood  clot  or 
has  undergone  extensive  calcareous  degeneration. 

Practitioners  unfamiliar  with  the  appearance  of  intraocular  growths 
sometimes  mistake  glioma  for  congenital  cataract  and  delay  treatment 
until  the  infant  is  older.  This  fatal  error  is  avoided  by  the  knowledge 
that  congenital  cataract  lies  immediately  behind  the  pupil  and  possesses  a 


286 


THE    FUNDUS    OCULI. 


soft,  bluish-white  color,  while  glioma  is  deeply  situated,  far  behind  the 
pupil  and  has  a  whitish  cast. 

Bibliography, 

Treacher  Collins,  Researches,  London,  1896. 

Greeff,   Lehrb.  d.   s.    Path.   Anat.    (Orth.),  II.  Halfte.    I.    Theil.    1903.    Deutsch.    m. 
Woch.  XXII,  1896.  D.  Bail.  u.  d.  Wesen  d.  Glioma,  Deut.  m.  Woch.  1896,  327. 
Parsons,  Path,  of  the  Eye,  vol.  II,  1905. 
SchobI,  Norris  and  Oliver,   III,  1898,  554. 
Virchovv,   Die   Krankhaften    Geschwulste,   Bd.   II,   151. 
Wintersteiner,  D.   Xeuroepithel.  Ret.  1897. 

The  following  table  is  applicable  only  to  the  early  stages  of  glioma 
of  the  retina,  at  which  time  the  diagnosis  Is  extremely  difficult  and  often 
impossible : 


GLIOMA. 

PSEUDOGLIOMA. 

History. 

May  attack  several  children  in 
a    family.      Not    necessarily    pre- 
ceded by  sickness  or  injury. 

No  family  history  of  eye 
tumor.  Preceded  by  some  infec- 
tions  fever   or  1 y   injury. 

Age. 

Usually    discovered    before    the 
third   year.      Probabilities    against 
its    development    after    fifth   year. 

May   occur    at    any   age. 

Anterior     Chamber 
and  Iris. 

Anterior  chamber  usually  clear. 
Iris  is  normal   and  free  from  ad- 
hesions. 

Iritic  adhesions,  atrophy  of 
iris,  hxpopyon  or  deposits  on 
lens  or  cornea,  indicate  an  in- 
fiammatery  process  rather  than 
glioma. 

Pupillary    Reactions. 

Early  glioma  affects  only  a  lim- 
ited area  of  retina ;    consequently, 
the  eye  retains  good  light  percep- 
tion   and    pupillary    reactions    are 
normal. 

Metastatic  retinitis  usually  in- 
volves the  entire  retina,  in  which 
case  all  light  perception  }nay  he 
lost  and  there  is  no  flircct  pupil- 
lary  reaction. 

Tension. 

Eye     tension      normal      or     in- 
creased. 

Eye  tension  frequently  sub- 
normal. 

Detachment. 

Glioma    often    is     accompanied 
by    simple    detachment    of    retina, 
which      trembles     on      movement. 
Therefore,    a    tumor    in    infants, 
accompanied     1  y     simple     detach- 
ment, probably  is  glioma. 

In  metastatic  endophthalmitis 
the  entire  retina  usually  is  in- 
filtrated with  exudates  which 
render  it  rigid  and  immovable 
when   the   eye   is   moved. 

INTRAOCULAR    AND    ORBITAL    TUMORS.  287 

Tl^MORS  OF  THE  CiLLXRY  BoDY.  Sarcoma.  Sarconia  of  the  ciliary 
body  usually  is  an  extension  of  choroidal  sarcoma.  Primary  sarcoma  of 
the  ciliary  body  rarely  assumes  the  mushroom  shape  so  frequent  in  sar- 
coma of  the  choroid,  but  forms  a  rounded  tumor  with  a  broad  base  which 
tends  to  grow  forward  into  the  iris  and  posterior  chamber  rather  than 
backward  into  the  choroid.  As  ciliary  sarcoma  advances  it  subluxates  and 
deforms  the  lens  but  shows  little  tendency  to  invade  it   (Pig.  222). 

The  so-called  ring  sarcoma  of  the  ciliary  body  is  very  rare  (Fig.  201 ) . 
It  usually  infiltrates  and  replaces  the  entire  ciliary  body  before  forming 
marked  tumefactions.  These  growths  are  of  relatively  low  malignancy 
and  may  exist  many  years  before  exciting  suspicion  as  to  their  real  char- 
acter. The  diagnosis  of  ring  sarcoma  is  difficult.  Transillumination  is 
of  no  assistance  unless  the  neoplasm  has  formed  local  tumefactions.  In 
the  late  stage  the  iris  may  contain  nodules  or  show  increased  pigmenta- 
tion. Usually,  however,  it  is  only  swollen  and  changed  in  color  (Fuchs). 
If  the  ciliary  processes  are  not  destroyed  glaucoma  finally  develops.  Histo- 
logically, these  growths  are  endotheliomata  (pages  271-273). 

Sarcomas  which  originate  in  the  anterior  portion  of  the  choroid  and 
extend  to  the  ciliary  body  possess  all  the  characteristics  of  choroidal 
sarcoma. 

Strrf.ogr AM  78.  Sarcoma  of  Choroid  and  Cillxrv  Body.  Left 
fundus  oculi  of  a  man,  28  years  of  age.  V^ision  in  the  left  eye  has  recently 
fallen  to  counting  fingers.  No  pain.  Tension  slightly  minus.  Externally, 
the  eye  is  not  reddened,  but  a  single  greatly  dilated  and  tortuous  anterior 
ciliary  vein  crosses  the  sclera  on  the  nasal  side.  The  ophthalmoscope 
reveals  a  very  flat  detachment  of  the  retina  in  the  macular  region.  At 
the  anterior  limits  of  the  ophthalmoscopic  field  on  the  nasal  side,  the 
posterior  edge  of  a  light  colored,  slightly  nodular  tumor  is  seen.  The 
surface  of  the  mass  is  traversed  by  blood  vessels  which  pursue  a  course 
the  reverse  of  the  retinal  vessels,  /.  e.,  from  before  backward.  The  pos- 
terior border  of  the  tumor  merges  into  loose  folds  of  detached  retina. 
The  detachment  presents  a  dark  color  owing  to  its  position  which  prevents 
illumination  by  the  choroidal  reflex.*  Transillumination  of  the  anterior 
nasal  region  corresponding  to  the  intraocular  mass,  casts  a  shadow  on  the 
pupil.     Diagnosis  is  sarcoma  of  the  choroid  and  ciliary  body. 


*  In  this  stereogram  the  tumor  is  situated  far  forward  in  the  eye,  and  when  it  is 
brought  into  focus  with  the  ophthahnoscope,  all  details  in  the  posterior  part  of  the  fundus 
are  lost. 


288  THE    FUNDUS    OCULI. 

The  eye  was  enucleated  and  found  to  contain  a  poorly  pigmented 
sarcoma    (Pig.  223). 

Conglomerate  tubercle  of  the  ciliary  body  may  simulate  sarcoma.  It 
differs,  however,  in  its  tendency  to  invade  all  structures  that  lie  in  its  path 
including  the  iris,  lens  (Fig.  224)  and  sclera.  In  ciliary  tubercle  sec- 
ondary deposits  usually  form  in  the  iris  and  excite  a  low  grade,  plastic 
Inliammation.  In  other  cases  conglomerate  tubercle  ci  the  ciliary  body  may 
attain  large  size  and  perforate  the  globe  without  exciting  the  slightest 
local  reaction   (Fig.  225). 

DiACJNOSls.  Tuberculosis  of  the  ciliary  body  usually  occurs  at  an 
earlier  age  than  sarcoma.  In  suspicious  cases  a  tuberculin  injection  may 
be  employed  for  diagnostic  purposes  and  a  positive  reaction  in  the  eye  is 
proof  that  the  process  is  tubercular. 

Metastatic  carcinoma  may  appear  in  the  ciliary  body.  In  a  case  which 
I  examined  microscopically  for  Dr.  J.  Dancan,  the  tumor  cells  extended 
backward  infiltrating  the  choroid,  thus  following  the  line  of  least  re- 
sistance   (Fig.   226). 

The  diagnosis  of  metastatic  ciliary  carcinoma  rests  upon  the  knowledge 
of  carcinoma  elsewhere  in  the  body. 

Glioma  has  been  known  to  originate  in  the  pars  ciliaris  retinae,  although 
in  the  great  majority  of  cases  ciliary  glioma  is  secondary  to  a  primary 
growth  in  the  retina.  Glioma  is  the  malignant  tumor  of  infancy  and  does 
not  enter  into  the  differential  diagnosis  of  intraocular  growths  in  adults. 

A  case  of  metastatic  hypernephroma  of  the  ciliary  body  has  been  re- 
ported by  Burton  Chance*  who  permitted  me  to  examine  microscopic  slides 
of  the  growth  (Fig.  227).  The  diagnosis  depends  upon  the  known 
presence  of  hypernephroma. 

Cysts  may  form  in  the  pars  ciliaris  retiucP  of  degenerated  eyes  by 
separation  of  its  layers  (Fig.  171).  They  occur  also  in  eyes  which  appear 
otherwise  normal,  especially  in  old  people  (Fig.  228).  They  should  be 
carefully  differentiated  from  melanosarcoma  by  means  of  transillumination. 

Sometimes  the  uveal  pigment  on  the  ciliary  processes  forms  large 
melanotic  masses  which  might  be  mistaken  for  malignant  growths  (Fig. 
229).  The  benign  melanotic  masses  which  I  have  seen  present  a  scintil- 
lating surface  when  viewed  with  the  ophthalmoscope.  Owing  to  their 
separation  from  the  sclera  they  do  not  cast  a  shadow  in  transillumination. 

I  once  microscopically  examined  an  eye  enucleated  for  supposed  sar- 
coma of  the  ciliary  body  which  on  section  was  found  to  contain  no  sar- 


*  Chance,  Jour.  Am.   Med.  Assn.,  Feb.  9,  1907,  475. 


Fig.  222 — Sarcoma  of  the  Cil- 
iary Body.  Tlie  Tumor  has  De- 
formed  and    Luxated   the    Lens. 


iMg.  ■22-3. 


Fig.  223 — Poorly  Pigmented  Sarcoma  of  the 
Choroid  and  Ciliary  Body. 


Fig.    224 — Conglomerate     Tubercle     of 
the  Ciliary  Body  Invading  the  Lens. 


Fig.  i;ii4. 


Fig.  225  —  C  o  n  - 
glomerate  Tubercle 
of  the  Ciliary  Body 
perforating  the 
Sclera. 


Fig.  225. 


Fig.  226. 


Fig.   227 — M  etastatic   Hydrone- 
phroma  of  the  Ciliary  Body. 


Fig.    226— Metastatic    Carcinoma   of 
the   Ciliary   Body. 


Fig.  227. 


Fig.  228. 
Fig.  228— Cyst  of  the  Pars  Ciliaris  Retinae. 


Fig.  229. 

Fig.  229— Masses  of  Uveal  Pigment  Simulating  Malignant  Growths  of  the  Ciliary 

Body.  ^ 


Fig.  230 — Telangiectasis  of 

the      Ciliary     Body.        Large 

Cystic   Spaces   also   exist  be- 

eath    the    Epithelial    Layer 


FiR.  2.30. 


Fig.  231 — Same  as  Fig.  230. 
The  Mesh  of  Blood  Vessels 
is  covered  by  Dense  Masses 
of  Uveal  Pigment,  forming 
Melanomata. 


Fig.  2.31. 


•v^ 


INTRAOCULAR    AND    ORBITAL    TUMORS.  289 

coma  but  an  angiomatous  formation,  probably  congenital.  The  clinical 
appearance  was  remarkably  deceptive.  The  subject  was  a  man,  46  years  of 
age.  Myopia  of  7.  D.  in  each  eye.  Recent  loss  of  vision  in  the  left  eye. 
Ophthalmoscope  showed  extensive,  temporal  detachment  of  the  retina  in- 
cluding the  macula  (Fig.  120).  Immediately  in  front  of  the  detachment 
and  connected  with  the  ciliary  body,  was  a  nodular  black  mass,  containing 
blood  vessels.  This  case  occurred  before  transillumination  was  employed 
in  eve  diagnosis.  The  eye  was  enucleated.  Microscopic  examination 
showed  that  the  tumor  seen  with  the  ophthalmoscope  was  formed  by  the 
long  ciliary  artery  which,  on  entering  the  ciliary  processes,  immediately 
broke  up  into  a  telangiectatic  m.esh  of  vessels  densely  covered  with  uveal 
pigment  which  in  places  formed  melanomata  (Figs.  230-231).  In  ad- 
dition, several  large  cystic  spaces  existed  beneath  the  epithelial  layer.  The 
detachment  of  the  retina,  which  caused  the  loss  of  vision  and  was  supposed 
to  be  due  to  the  ciliary  tumor,  proved  to  be  simple  in  its  nature  and  such 
as  is  common  in  myopic  eyes. 

Tumors  "of  the  Orbit  and  Optic  Nerve.  Extraocular  tumors 
may  induce  changes  in  the  fundus  of  the  eye  consisting  of  optic  neuritis, 
optic  atrophy  and  secondary  hypermetropia  from  pressure  on  the  ocular 
walls. 

Orbital  Tumors  may  be  malignant  or  benign. 

Di'RMOiD  Cysts  are  the  most  common  of  all  growths  originating  in 
the  orbit.  They  are  always  congenital,  although  they  may  be  so  small  at 
birth  as  to  escape  observation.  The  great  majority  are  situated  in  front 
of  the  eye  and  do  not  cause  exophthalmus  unless,  as  in  some  cases,  they 
extend  deeply  into  the  orbit. 

Fluid  in  Tenon's  Capsule  or  on  the  tendons  of  muscles  may  simu- 
late cysts. 

Hydatid  Cysts  usually  occur  in  the  young,  two-thirds  of  all  cases 
occurring  between  the  ages  of  11   and  21  years   (Neisser). 

Inflammatory  Tumors  and  bulging  inward  of  the  orbital  walls 
are  due  to  disease  or  tumors  in  the  adjacent  nasal  sinuses. 

Granulations  and  Polypi  in  the  orbit  are  connected  with  bone 
disease,  usually  originating  in  adjoining  nasal  cavities. 

Primary  Exostoses  of  the  orbit  usually  spring  from  the  upper  and 
inner  angle,  although  they  may  grow  from  any  portion  of  the  bony 
wall.  They  are  composed  of  an  extremely  dense,  bony  shell  containing 
a  center  of  spongy  bone  (ivory  exostosis)  ;  rarely,  they  are  partly  car- 
tilaginous.    When  situated  in  the  sphenoidal  fissure,  the  optic  nerve  is 

19 


290  THE    FUNDUS    OCULI. 

compressed  and  atrophy  results.  Osteoma  may  invade  the  orbit  from 
without.  Such  cases  should  be  carefully  differentiated,  for  the  reason 
that  primary  tumors  situated  superficially  are  easily  removed,  whereas  it 
is  highly  dangerous  to  operate  upon  those  which  have  extended  into  the 
orbit  from  adjoining  cavities. 

Simple  Angioma  (telangiectasis)  and  Cavernous  Angioma  both 
occur  in  the  orbit.  Simple  angiomata  are  congenital  growths,  situated  in 
the  lids,  whence  they  extend  into  the  orbit.  They  are  not,  as  a  rule,  en- 
capsulated. Cavernous  angiomata  develop  in  the  orbit.  Thev  possess 
a  thick  fibrous  capsule,  lined  with  endothelium  and  are  crossed  by  trabeculae, 
the  structure  resembling  erectile  tissue.  They  may  contain  phleboliths. 
Bruit  and  pulsation  are  not  present  in  these  tumors. 

Lymphangiomata  seldom  occur  in  the  orbit. 

LiPOMATA  are  reported  as  occurring  in  the  orbit  but  such  diagnoses 
must  be  accepted  with  caution  inasmuch  as  the  amount  of  adipose  tissue 
normally  present   varies  widely  within   physiologic   limits. 

T>YMPHOMATA  occur  in  the  orbit  under  two  forms,  (  1 )  leukemic 
and  pseudoleukemic  deposits,  both  of  which  are  associated  with  disease 
of  the  blood  and  blood  forming  organs;  (2)  round  celled  growths  re- 
sembling sarcoma  but   apparently  nonmal'gnant. 

Neuromata,  i.  e.,  growths  consisting  of  newly-formed  nerve  ele- 
ments, and  neurofibromata  have  been  observed  in  the  orbit.  Plexiform 
neuroma  (Elephantiasis  Neuromatodes,  Cirsoid  Neuroma)  occurs  In  the 
orbit  very  rarely. 

Fibromata  may  grow  from  the  periosteum,  sheaths  of  tendons  and 
dural  sheath  of  the  optic  nerve.  In  diagnosticating  fibroma  of  the  orbit, 
the  tendency,  of  other  growths,  especially  angiomata,  to  undergo  fibrous 
hyperplasia  must  be  borne  in  mind. 

Carcinoma  does  not  occur  primarily  in  the  orbit  but  invasion  may 
occur  from  the  skin,  conjunctiva,  lachrymal  gland  and  from  metastasis 
in  the  choroid. 

Sarcoma  may  originate  in  the  orbit  although  in  the  great  majority 
of  cases  it  is  an  extension  or  metastasis  from  the  choroid. 

Glioma  of  the  Orbit  is  always  a  secondary  deposit  from  glioma 
of  the   retina. 

Cylindroma  (endothelioma)  forms  encapsulated  tumors  in  the 
orbit.     They  are  of  very  low  malignancy. 

The  prognosis  after  operation  in  orbital  tumors  is  far  more  favorable 
when  the  tumor  is  encapsulated  than  when  it  Is  diffuse. 


Fig.  232 — Orbital  Tumor  causing 
Localized  Hypermetropia  by  Pressure 
upon,  and  Thickening  of,  the  Sclera. 
S,  sclera;  T,  tumor. 


Fig.  232. 


Fig. 


-Tumor  of  the  Optic  Nerve. 


Fig.  233. 


Fig.  234 — The  so-called  Glioma  of  the 
Optic  Nerve,  showing  its  development  from 
the    Connective    Tissue    Septa. 


Fig.  234. 


INTRAOCULAR    AND    ORBITAL    TUMORS.  291 

Lachrymal  Gland  Tumors  are  very  rare.  Frequently,  they  are 
mixed  growths.  Carcinoma,  sarcoma,  myxoma,  myxadenoma,  m^Tcosar- 
coma,  enchondroma,  osteochondroma,  osteoma,  fibroma,  adenoma,  adeno- 
angioma,  cylindroma,  conglomerate  tubercle  and  hydatid  cysts  have  been 
reported  as  occurring  in  the  lachrymal  gland. 

Protusion  of  the  eyeball  (exophthalmus)  is  the  most  prominent 
symptom  of  orbital  tumor.  If  situated  without  the  muscle  cone,  the  eye 
is  pushed  to  one  side  according  to  the  situation  of  the  growth.  Unlike 
tumors  of  the  optic  nerve,  orbital  growths  do  not  produce  early  blindness; 
consequently,  diplopia  usually  is  present.  A  tumefaction  at  the  apex  of 
the  orbit,  however,  may  destroy  sight  from  pressure  on  the  optic  nerve. 
Pressure  of  an  orbital  growth  on  the  eyeball  may  push  the  walls  inward 
or  cause  the  sclera  to  thicken,  in  which  case  the  ophthalmoscope  vvill 
show  a  localized  hypermetropia   (Fig.  .232). 

Tumors  of  the  Optic  Nkrve.  Tumors  of  the  optic  nerve  are 
divided  into  two  classes,  intradural  and  extradural.  The  latter  are  very 
rare  and  develop  around  the  nerve  outside  the  sheaths.  Strictly  speak- 
ing, only  those  growths  which  develop  within  the  dural  sheath  should 
be  considered   as  primary  tumors  of  the  nerve. 

Intradural  tumors  of  the  optic  nerve  mostly  begin  In  early  life  or 
are  congenital.  Out  of  85  cases  collected  by  Byers,  S2  appeared  before' 
the  25th  year.  They  are  of  slow  development  and  low  malignancy. 
As  a  rule,  they  extend  backward  from  the  entrance  of  the  central  vessels, 
Ifeaving  10  mm.  to  12  mm.  of  the  optic  end  of  the  nerve  free.  They  may 
also  extend  forward  and  press  against  the  globe. 

Optic  nerve  tumors  vary  in  size  from  a  slight  enlargement  to  the 
size  of  an  egg  (Fig.  233).  In  a  large  proportion  of  cases  they  are  con- 
nected with  an  intracranial  extension  which  is  not  removed  with  the  tumor, 
and  the  continued  growth  of  which  ultimately  destroys  life.  They  are 
usually  elastic  or  of  soft  consistency  and  may  contain  large  cystic  spaces. 
Histologically,  they  belong  to  the  connective  tissue  group.  The  most 
characteristic  nerve  tumor  is  m^Tcoma  or  myxosarcoma.  Endotheliomata 
also  are  common.  When  these  tumors  contain  chalky  concretions  they 
are  called  psamomata.  Fibroma  and  fibrosarcoma  are  rare.  Greeff  says 
the  so-called  glioma  of  the  nerve  develops  from  the  connective  tissue 
septa  of  the  nerve,  the  neuroglia  taking  no  part  in  their  formation  (Fig. 
234).  Were  the  tissue  of  these  neoplasms  proliferating  neuroglia  they 
would  react  to  selective  glia  stains,  which  they  fail  to  do.  The  term 
"  neuroma  "  when  applied  to  these  tumors  also  is  a  misnomer,  because 


292  THE    FUNDUS    OCULI. 

the  optic  nerve  is  brain  tissue,  which  does  not  proliferate.  Greeff  applies 
Cohnheim's  theory  to  the  development  of  intradural  tumors.  He  believes 
that  when  the  central  vessels  enter  the  fetal  cleft  in  the  nerve,  a  certain 
number  of  embryonic  cells  are  carried  along,  which  persist  and  ultimately 
proliferate,  forming  a  tumor  of  the  nerve. 

The  exophthalmus  of  optic  nerve  tumor  is  characteristic.  The  eye 
is  thrust  directly  forwards  with  little  or  no  lateral  displacement.  Mo- 
tility is  not  disturbed  until  exophthalmus  is  extreme.  The  eyeball  cannot 
be  pushed  back  into  its  normal  position.  Blindness  occurs  early,  often 
before  the  exophthalmus  attracts  attention,  in  which  event  the  patient 
has  no  diplopia.  Usually,  there  is  first  choked  disc  followed  by  postneuritic 
atrophy.  The  central  vessels  in  the  nerve  are  destroyed  but  this  occurs 
so  gradually  that  the  retinal  circulation  is  continued  by  the  new-formed 
vessels  in  the  tumor  without  inducing  either  ischemia  or  stasis.  The 
development  of  new  vessels  around  the  ocular  end  of  the  nerve  may 
explain  why  circulation  persists  in  the  retina  after  removal  of  nerve  and 
tumor.  When  exophthalmus  is  extreme  the  lids  cannot  cover  and  pro- 
tect the  cornea  which  becomes  dry,  dessicated  and,  finally,  ulcerated  (kera- 
titis e  lagophthahno) .  In  this  condition  the  lower  half  of  the  cornea 
suffers,  chiefly  because  this  portion  is  uncovered,  especially  during  sleep 
when  the  lids  are  at  rest  and  the  cornea  directed  upward.  Exceptionally, 
pressure  of  the  tumor  may  advance  the  posterior  pole  of  the  eye,  thus  pro- 
ducing a  secondary  hypermetropia. 

Stereogram  79.  Optic  Atrophy  and  Secondary  Hypermetropi-a 
FROM  Tumor  of  the  Optic  Nerve.*  Left  eyeground  of  apparently 
healthy  girl,  14  years  of  age.  Left  exophthalmus  first  attracted  attention 
two  months  ago,  at  which  time  she  had  transient  diplopia.  At  present, 
the  eye  is  blind.  Exophthalmus  is  marked,  the  displacement  being  directly 
forward.  The  eye  cannot  be  restored  by  pressure  to  its  normal  position. 
No  pain.  Motility  is  unimpaired.  The  cornea  and  media  are  transparent. 
The  optic  disc  presents  the  dead  white  color  of  postneuritic  atrophy. 
Retinal  vessels  normal.  The  entire  posterior  pole  is  elevated  above  other 
parts  of  the  fundus.  The  center  of  this  area  has  an  elevation  of  2  mm. 
(6.  D.)  ;  the  periphery  slopes  gradually  away  to  the  level  of  the  normal 
fundus.  The  projection  does  not  resemble  detached  retina  but  gives  one 
the  impression  that  the  posterior  walls  of  the  globe  are  pushed  inward. 
It  is  crossed  by  several  horizontal  light  streaks,  presumably  caused  by 
inequalities  in  the  surface. 


*  Patient  was  seen  through  courtesy  of   Dr.  Frank  Van  Fleet  who  reported  case  in 
Medical  Record,  June  27,  1908. 


INTRAOCULAR    AND    ORBITAL    TUMORS.  293 

A  tumor  of  the  optic  nerve  was  discovered  and  removed  by  the 
Kronlein  operation. 

Diagnosis.  A  tumor  of  the  nerve  produces  anterior  displacement 
of  the  eye;  a  tumor  of  the  orbit  produces  lateral  displacement.  In  nerve 
tumors  motility  of  the  eye  is  good;  in  orbital  tumors  it  is  defective.  The 
eye  is  blind  in  tumor  of  the  nerve,  but  some  vision  is  retained  in  orbital 
growths;  therefore,  diplopia  exists  in  the  latter  condition  but  not  in  tumor 
of  the  nerve  except,  possibly,  in  the  early  stage.  Age  is  an  important 
factor  in  differentiating  these  conditions,  inasmuch  as  about  40  per  cent  of 
intradural  nerve  tumors  develop  before  the  tenth  year.  In  exophthalmus 
due  to  solid  tumor  the  eye  cannot  be  pushed  backward  into  its  normal 
position,  differing  in  this  respect  from  exophthalmic  goitre  and  telangiec- 
tasis of  the  orbit  in  which  the  displaced  eve  can  temporarily  be  reduced 
by  pressure.  The  secondary  hypermetropia  presented  by  the  above  case 
is  unusual  in  tumor  of  the  nerve. 

Bibliography. 

Byers,  Stud,  from  R.  Victoria  Hosp.  Montreal,  1901. 

Neisser.  Die  Echinokockenkrank.,  Berlin,  1877. 

Greef,  Orth's  Lehrb.  d.  spez.  Path.  Anat.  II  1,  Theil,  p.  450. 


INDEX. 
A 

PAGE 

Albinism  of  the  Eyeground,  acquired   (Stereogram  26) 55 

Albinism  of  the   Eyeground,  congenital   (Stereogram   15) 33 

Albuminuric  Retinitis  (see  Retinitis)    81 

Amaurosis,   toxic    208 

Amaurosis,   uremic    87 

Amaurotic  Family  Idiocy  (Stereogram  57) 200 

Amblyopia,   alcoholic    209 

Amblyopia,    from  iodoform    210 

Amblyopia,    from  quinine 210 

Amblyopia,    from  salicylic  acid    210 

Amblyopia,   from  tobacco    209 

Amblyopia,    from  wood  alcohol    208 

Amblyopia,   toxic    208 

Atrophia  Gyrata  Choroidae  et  Retinae 181 

Atrophy  of  the  Choroid   (see  Choroid)    55 

Atrophy  of  Optic  Nerve  (see  Optic  Nerve)    231 

B 

Buphthalmus    264 

C 

Canal  of  Cloquet  (Stereogram  14)    32 

Choked   Disc    (Stereograms  63-64)    220 

Choked   Disc,   causes  of    221 

Choked   Disc,   in  brain  tumor 222-224 

Choked   Disc,  theories  of 220 

Choriocapillaris    12 

Choroid 11 

Choroid,   affections  of 37 

Choroid,   atrophy  of  (Stereogram  26)    5S 

Choroid,   congenital  anomalies  of 20 

Choroid,   conglomerate  tubercle  of   51 

295 


296 


INDEX. 


Choroid,   miliary  tuberculosis  of    51 

Choroid,   rupture  of    (Stereogram  28)    59 

Choroid,   sarcoma  of   (Stereogram  74-75 )    270 

Choroid,   sclerosis  of    54 

Choroid,  tuberculosis  of   (Stereogram  23)    48 

Choroid,  tumors  of    270 

Choroid,   vascular  degeneration  of 54 

Choroidal  Ring 18 

Choroiditis,   central   (Stereograms  19-21 )    42 

Choroiditis,   disseminated   (Stereograms   17-18)    37 

Choroiditis,   disseminated,   differentiation    39 

Choroiditis,   disseminated,   stage   of  cicatrization    (Stereogram  20)  .  43 

Choroiditis,   disseminated,   stage  of  repair    44 

Choroiditis,   disseminated,   symptoms   of    41 

Choroiditis,   exudative    37 

Choroiditis,   metastatic 215 

Choroiditis,   syphilitic    (Stereogram    19)     42 

Choroiditis,   suppurative    37—215 

Chorioretinitis,   diffuse,   atrophic  stage   (Stereogram  24)    52 

Chorioretinitis   Pigmentosa    (Stereogram  22)    46 

Chorioretinitis   Pigmentosa,  differentiation  from  retinitis  pigmentosa  48 

Ciliary  Body,   tuberculosis  of 288 

Ciliary  Body,   tumors  of    287 

Cilio-retinal  Arteries 15 

Circle  of  Zinn 16 

Coloboma,  Fuchs'  (Stereogram  10)    26 

Coloboma   of  the   Choroid   (Stereogram  6)    20 

Coloboma   of  the   Choroid,   differentiation  from  old  cicatrices 21 

Coloboma  of  the   Choroid,   pathogenesis 22 

Coloboma  of  Choroid  and  Optic  Nerve   (Stereogram  7)    21 

Coloboma  of  the   Macula   (Stereogram  8)    23 

Coloboma  of  the  Optic  Nerve  and  Sheath   (Stereogram  9)    24 

Coloboma   of  the   Optic  Nerve   and   Sheath,    differentiation    from 

glaucoma   26 

Colloid  Excrescences  (Stereogram  27)    57 

Commotio  Retinas  (Stereogram  59)    205 

Crescent,   inferior    (Stereogram    10)    26 

Crick  Dots   (Stereogram  16)    35 

Cyanosis  Retinas   (Stereogram  58)    204 


INDEX.  297 

D 

PAGE 

Degenerations  of  the  Retina 179 

Detachment  of  the  Retina    (see  Retina)    159 

Diabetic  Retinitis    (see  Retina)    94 

Diaphanoscopy    7 

Direct  Method  of  Ophthalmoscopy  (Stereogram  1 )    3 

Disseminated  Choroiditis  (see  Choroid)    37 

E 

Embolism  of  the  Central  Artery  (see  Retinal  Vessels)    105 

Endogenous  Ophthalmitis 215 

Examination   of  the  Eye    1 

Examination  of  the  Eye,   by  determination  of  the  visual  fields.  ...  6 

Examination  of  the   Eye,  by  tonometry    9,  10 

Examination  of  the  Eye,   by  transillumination    7 

Examination   of  the   Eye,   with   oblique    illumination    5 

Examination  of  the  Eye,   with  the  ophthalmoscope    1 

F 

Fuchs'  Coloboma   (Stereogram   10)    26 

Fundus  Oculi,   normal    (Stereogram  2)    11 

Fundus  Oculi,   senile  changes  in  (Stereogram  27)    57 

Fundus  Oculi,   negroid    (Stereogram  3)    14 

G 

Glaucoma    251 

Glaucoma,   absolutum    253 

Glaucoma,   acute 252 

Glaucoma,  causes  of 257  et  seq. 

Glaucoma,   chronic    254 

Glaucoma,   degenerativum    254 

Glaucoma,   fulminans    254 

Glaucoma,  hemorrhagic   (Stereogram  73)    255-265 

Glaucoma,   infantile   (buphthalmus)    264 

Glaucoma,   ophthalmic  picture  of   (Stereogram  72)    255 

Glaucoma,   pathogenesis  of 257 

Glaucoma,  pathology  of 262 


298  INDEX. 

PAGE 

Glaucoma,   primary    251 

Glaucoma,   secondary    260 

Glaucoma,   simple   (Stereogram  72) .254—256 

Glioma   of  the   Retina  (Stereogram  76)    278 

Glioma  of  the   Retina,   diagnosis  of    ,283-286 

Glioma   of  the   Retina,   morbid  anatomy  of    28  3 

Gunn's  Dots   (Stereogram  16)    3S 

H 

Hemorrhage,   intravitreous,  recurrent 77 

Hemorrhage,   intravitreous,   recurrent,  causes  of 78 

Hemorrhage,   preretinal    (Stereogram  33)    76 

Hemorrhage,   retinal 71 

Hemorrhage,   retinal,    etiology   of    73 

Hemorrhage,   subhyaloid    (Stereogram  33)    76 

Hemorrhagic  Albuminuric  Retinitis  (Stereogram  35)    90 

Hemorrhagic  Diabetic  Retinitis   (Stereogram  38)    95 

Hemorrhagic  Leukemic  Retinitis  (Stereogram  39)    98 

Hemorrhagic  Neuroretinitis  (Stereogram  47)    139-142 

Hereditary  Macular  Anomaly    , 197 

Hereditary  Optic  Atrophy 235 

Hyaloid  Artery,  persistent  (Stereogram  14)    32 

I 

Indirect  Method  of  Ophthalmoscopy 4 

Inferior  Optic  Crescent   (Stereogram  10)    26 

Intraocular  Pressure    9 

Intravitreous  Hemorrhage    77 

L 

Lamina  Vitrea 12 

Leukemic  Retinitis    (see  Retina)    96 

M 

Macula  Lutea    13 

Macula,   Coloboma  of  (Stereogram  8)    23 

Maculo-cerebral   Degeneration    (familial)    (Stereograms   55-56)  ...  191 


INDEX.  299 

PAGE 

Maculo-cerebral   Degeneration,  macular  type    192-194 

Maculo-cerebral   Degeneration,   maculo-cerebral  type    .....191-192-193 

Maculo-cerebral   Degeneration,   differentiation    197 

Maculo-cerebral   Degeneration,   etiology    196 

Maculo-cerebral   Degeneration,   pathology    195 

Medullated  Nerv^e  Fibers   (Stereogram  11)    27 

Metastatic  Ophthalmitis    (Stereogram   77)    215 

Myopia    244 

Myopia,   anatomical  changes  in    246 

Myopia,   etiology  of    244 

Myopia,   non-progressive    (Stereogram  70)    246 

Myopia,   progressive  (Stereogram  71)    248 

Myopia,  varieties  of 244 

N 

Neuritic  Optic  Atrophy  (Stereogram  69)    236 

Neuritis,  Optic  (Stereograms  31,  35,  36,  6S,  66)    220 

Neuritis,   Optic,   in  cerebral  sinus  thrombosis   (Stereogram  66)  ...  .  228 

Neuritis,   Optic,   in     disease  of  nasal  sinuses   (Stereogram  6S) 225 

Neuritis,   Optic,   with  albuminuric  retinitis  (Stereogram  36) 91 

Neuro-retinitis,  hemorrhagic 138-139-142 

o 

Oblique,  or  Focal  Illumination 5 

Ophthalmoscope    1 

Opaque  Nerve  Fibers   (Stereogram   11)    27 

Optic   Atrophy    23 1 

Optic  Atrophy,   following  occlusion  of  central  vessels (Stereogram44)  133 

Optic  Atrophy,   following  injury  of  orbit 236 

Optic  Atrophy,   hereditary    235 

Optic  Atrophy,   in  disease  of  the  pituitary  body 235 

Optic  Atrophy,   in  lateral  sclerosis  of  the  cord 235 

Optic  Atrophy,   in  multiple  sclerosis   (Stereogram  68)    233 

Optic  Atrophy,   in  paresis    235 

Optic   Atrophy,   in  syringomyelia    235 

Optic   Atrophy,   in  tabes   (Stereogram  67)    231 

Optic  Atrophy,   post-neuritic  (Stereogram  69)    236 

Optic  Atrophy,   retinitic   (Stereograms  22-53)    180 


300  INDEX. 

PAGE 

Optic  Atrophy,   simple    23 1 

Optic  Crescent,  inferior  (Stereogram  10)    26 

Optic  Nerve,   anatomy  of    18 

Optic  Nerve,   atrophy  of    231 

Optic  Nerve,   compression  of,  in  arteriosclerosis    239 

Optic  Nerve,  tumors  of , 291 

P 

Papilledema    (Stereograms  63-64)    220 

Physiologic  Excavation  of  the  Disc  (Stereograms  5-40)    18 

Postneuritic  Optic  Atrophy   (Stereogram  69)    236 

Post-thrombotic  Changes  in  the  Fundus   (Stereogram  49)    156 

Prepapillary  Retinal  Artery   (Stereogram   12)    29 

Preretinal  Hemorrhage   (Stereogram  33)    _ 76 

Pseudoglioma   (Stereogram  77)    282 

Pseudoneuritis    (Stereogram   13)    30 

Pulsation  of  Retinal  Vessels    16 

Pulsation  of   Retinal  Vessels,   in  arteriosclerosis    62—64 

Punctate  Condition  of  the  Fundus  (Stereogram  16)    35 

Q 

Quinine  Amblyopia 210 

R 

Retina,   normal    13 

Retina,   angioid  streaks  in    ' 213 

Retina,   cyanosis  of   (Stereogram  58)    204 

Retina,   detachment  of  (Stereograms  50-51-52)    159 

Retina,   detachment  of,   diagnosis    164 

Retina,   detachment  of,   etiology    168 

Retina,   detachment  of,   incipient   (Stereogram  50)    160 

Retina,   detachment  of,   old   (Stereogram   52)    166 

Retina,   detachment  of,   prognosis    168 

Retina,   detachment  of,   recent   (Stereogram  51)    163 

Retina,   detachment  of,   serous    159 

Retina,   detachment  of,   symptoms    161-167 

Retina,   glioma  of  (Stereogram  76)    278-279 


INDEX. 


301 


Retina,   miscellaneous  affections  of 204 

Retina,   re-attachment    of    165 

Retina,   traumatic  edema  of   (Stereogram  59)    205 

Retinal   Hemorrhage    71 

Retinal   Hemorrhage,   etiology  of    73 

Retinal   Reflexes    (Stereogram   3)     14 

Retinal  Vessels,   normal    (Stereogram  4)    15 

Retinal  Vessels,   closure  of    105 

Retinal  Vessels,   closure  of  central   artery  (Stereog.  41,  42,  43,  45)  105 

Retinal  Vessels,   closure  of  central   artery,   anatomic   characters....  126 

Retinal  Vessels,   closure  of  central   artery,   clinical  history 132 

Retinal  Vessels,   closure  of  central   artery,   etiology    113 

Retinal  Vessels,   closure   of  central  vein    (Stereograms  46-47-48)  .  .  138 

Retinal  Vessels,   closure  of  central  vein,   etiology    140 

Retinal  Vessels,   closure  of  central  vein,   clinical  history 150 

Retinal  Vessels,   closure  of  central   vein,   complications 151 

Retinal  Vessels,   closure  of  central  artery  and  vein 156 

Retinal  Vessels,   sclerosis  of    (Stereograms  29-30)    62 

Retinal  Vessels,   sclerosis  of,   diagnosis    66 

Retinal  Vessels,   sclerosis  of,   etiology    63 

Retinal  Vessels,   sclerosis  of,   morbid    anatomy    68 

Retinal   Vessels,   sclerosis  of,   with  hemorrhages   (Stereogram  32)..  70 

Retinal  Vessels,   sclerosis  of,   with  optic  neuritis  (Stereogram  31 ).  .  69 

Retinitis,   Albuminuric  (Stereograms  35,  36,  37)    81 

Retinitis,  Albuminuric,   acute 86 

Retinitis,  Albuminuric,   differentiation  from  choroiditis    91-92 

Retinitis,   Albuminuric,   with  hemorrhages    90 

Retinitis,   Anglopathic    (Stereogram  34)     80 

Retinitis,   Circlnata    (Stereogram  40)    101—104 

Retinitis,   Diabetic   (Stereogram  38)    94 

Retinitis,   Leukemic   (Stereogram  39)    96 

Retinitis,   Metastatic    215 

Retinitis,   Pigmentosa  (Stereogram  53)    179 

Retinitis,   Pigmentosa  sine  Pigmento    181 

Retinitis,   Prollferans   (Stereogram  61)    211 

Retinitis,   Punctata  Albescens    (Stereogram  54)    181-189-190 

Retinitis,   Septic    52—217 

Retinitis,   Striata   (Stereogram  62) 213 


302  INDEX. 

PAGB 

Retinitis,  with  Pernicious  Anemia    98 

Retinitis,   with  Syphilis    100 

Retinitis,  with  Wood  Alcohol,  Poisoning   (Stereogram  60)    208 


S 

Sarcoma  of  the   Choroid    (Stereograms   74-75-78)    270 

Sarcoma   of  the   Ciliary  Body  (Stereogram  78)    287 

Sclera    11 

Sclerosis  of  the   Choroid    (Stereogram   25)    54 

Sclerosis  of  the   Retinal  Vessels  (see  Retinal  Vessels)    62 

Senile  Changes  in  the  Fundus  (see  Fundus)    57 

Septic  Retinitis   52-217 

Subhyaloid   Hemorrhage    (Stereogram   33)     76 

Suprachoroidea    11 

Staphyloma,   ciliary    263 

Staphyloma,   intercalary 263 

Staphyloma,   posterior,    in  myopia    247 

Staphyloma,   verum    247 

T 

Tabetic  Atrophy  of  the  Optic  Nerv^e  (Stereogram  67)    231 

Tay's   Disease    200 

Thrombosis  of  Central  Retinal  Vein   (Stereograms  46-47-48) 138 

Tobacco  Amblyopia    209 

Tonometer    10 

Tonometry,   digital    9 

Tonometry,   instrumental    10 

Toxic  Amblyopia  and  Amaurosis 208 

Transillumination    7 

Tuberculosis  of  the   Choroid    (see    Choroid)     48 

Tuberculosis,  of  the  Ciliary  Body   (see  Ciliary  Body)    288 

Tumors,   Intraocular    and    intraorbital    270 

Tumors,   of  the  ciliary  body 287 

Tumors,   of  the  choroid    270 

Tumors,   of  the  optic  nerve 291 

Tumors,   of  the  optic  nerve  causing  hyperopia    (Stereogram  79)  .  .  292 

1  umors,   of  the  orbit    289 


INDEX.  303 

u 

PAGE 

Uremic  Amaurosis   87 

V 

Vascular  Degeneration  of  the  Choroid  (Stereogram  25)    54 

Vitreous  Hemorrhage,   recurrent    77 

Vitreous   Hemorrhage,   recurrent,   causes  of    78 

Vitreous  Hemorrhage,   recurrent,  prognosis    79 

Z 

Zinn,   Circle  of    16 


